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1.
Acta Cytol ; 65(6): 541-548, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34569497

RESUMO

Myxoid leiomyosarcoma (MLS) is a rare variant of leiomyosarcoma, with most cases occurring in the uterus. A case of MLS arising in the periosteal region of the tibia, mimicking extraskeletal myxoid chondrosarcoma (EMC), is described. The evaluation included histological and cytological comparison with EMC. The patient was a 77-year-old man with a palpable mass at the anterior aspect of the right lower leg. After diagnosis by cytopathology and biopsy examination, a wide resection was performed. The resulting cytological smears were composed primarily of spindle-shaped tumor cells in a myxoid and hemorrhagic background. Histologically, the tumor showed abundant myxoid matrix and tumor cells proliferating in a cord-like to reticular pattern, exhibiting a lace-like arrangement that mimicked EMC. Although immunohistochemical findings suggested leiomyosarcoma, a diagnosis of EMC eventually was excluded by the lack of a split signal when assessed for a rearrangement of NR4A3 by chromogenic in situ hybridization. Despite histological similarity to EMC, characteristic cytological findings of EMC such as epithelioid structures with a cord-like pattern and chondroblast-like lacunar structures were not observed in the smears of this patient's MLS. We propose that cytopathological examination of bone and soft tissue lesions is useful as a diagnostic tool in similar cases. A total diagnostic workup, including clinical, radiographic, cytopathological, histopathological, and molecular findings, is needed to ensure an accurate final diagnosis and to reduce diagnostic error.


Assuntos
Neoplasias Ósseas/patologia , Condrossarcoma/patologia , Leiomiossarcoma/patologia , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/patologia , Tíbia/patologia , Idoso , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/genética , Neoplasias Ósseas/química , Neoplasias Ósseas/genética , Neoplasias Ósseas/cirurgia , Condrossarcoma/química , Condrossarcoma/genética , Proteínas de Ligação a DNA/genética , Diagnóstico Diferencial , Rearranjo Gênico , Humanos , Imuno-Histoquímica , Hibridização In Situ , Leiomiossarcoma/química , Leiomiossarcoma/genética , Leiomiossarcoma/cirurgia , Masculino , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/química , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/genética , Valor Preditivo dos Testes , Receptores de Esteroides/genética , Receptores dos Hormônios Tireóideos/genética , Tíbia/química , Tíbia/cirurgia
2.
Biomed Res Int ; 2021: 2106972, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34395610

RESUMO

Leiomyosarcoma is an uncommon soft tissue sarcoma that composed of malignant mesenchymal cells with distinct features of the smooth muscle lineage. Typically affects the uterus and gastrointestinal tract, it can rarely be seen in large blood vessels, lymphatic and glandular duts, the mesentery, the omentum, retroperitoneum, and limbs. Occurrence is particularly rare in the limb region. Retrospective study based on patient records and postoperative pathological histological features. Four patients with limb leiomyosarcoma that were operated between 2016 and 2020 were included, three of them arising in the subcutis of the thigh region and one in cubitus. Extend resection with satisfactory outcomes is reported. Pathological examination showed that masses were composed of a fascicular arrangement of hyperchromatic spindle-shaped cells, characterized by the proliferation of epithelioid cells with eosinophilic cytoplasm for epithelioid leiomyosarcoma. Leiomyosarcomas that arise in the soft tissue, although rare, should be differentiated from other lesions, such as neurilemoma, neurofibroma, liomyoma,lipomyoma, synoviosarcoma, rhabdomyosarcoma, malignant fibrous histiotoma, and malignant neurinoma.


Assuntos
Extremidades/patologia , Leiomiossarcoma/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Adulto , Idoso , Extremidades/diagnóstico por imagem , Extremidades/cirurgia , Feminino , Humanos , Leiomiossarcoma/diagnóstico por imagem , Leiomiossarcoma/patologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/patologia , Resultado do Tratamento , Adulto Jovem
3.
Br J Radiol ; 94(1125): 20210283, 2021 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-34289327

RESUMO

Leiomyomas are the most common benign tumors of the uterus. On the opposite side, leiomyosarcomas are rare malignant uterine tumors that account for a significant proportion of uterine cancer deaths. Especially when large and degenerated, leiomyomas and leiomyoma variants can have overlapping imaging characteristics with those of leiomyosarcomas. Although not always possible, it is paramount to be able to differentiate between leiomyomas and leiomyosarcomas on imaging, as the therapeutic management can differ. This pictorial review aims to familiarize radiologists with imaging features of leiomyomas and various types of leiomyoma degeneration and variants, together with their pathology correlates.


Assuntos
Leiomioma/diagnóstico por imagem , Leiomioma/patologia , Leiomiossarcoma/diagnóstico por imagem , Leiomiossarcoma/patologia , Imageamento por Ressonância Magnética/métodos , Neoplasias Uterinas/diagnóstico por imagem , Neoplasias Uterinas/patologia , Diagnóstico Diferencial , Feminino , Humanos , Sistemas de Informação em Radiologia , Útero/diagnóstico por imagem , Útero/patologia
4.
Gynecol Obstet Invest ; 86(4): 408-414, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34284401

RESUMO

OBJECTIVE: The aim of this study was to evaluate the impact of initial surgical treatments and surgical re-exploration on the oncological outcomes of patients with incidentally diagnosed uterine leiomyosarcoma (uLMS). DESIGN: A retrospective analysis of 87 patients who underwent re-exploration for incidentally diagnosed uLMS at the Fudan University Shanghai Cancer Center was performed. METHODS: Kaplan-Meier and Cox proportional hazards regression models were used for analyses. RESULTS: The median age (range) at diagnosis was 49 (23-76) years. The median disease-free survival (DFS) and overall survival (OS) were 15.1 and 56.7 months, respectively. The Kaplan-Meier curves and log-rank tests showed the median DFS was longer in patients with restaging operation (N = 30) than without (N = 55) (p = 0.017) but no significant difference in median OS (p = 0.142). In patients who underwent myomectomy/subtotal hysterectomy, 5 patients (28.5%) had remnant sarcoma during re-exploration with complete uterine removal, and re-exploration was associated with longer DFS (p = 0.038) and OS (p = 0.042). LIMITATIONS: The limitation of this study is its retrospective observational design with a limited number of patients. CONCLUSION: Reoperation with total hysterectomy after incomplete surgery may be helpful in patients with incidentally diagnosed uLMS at the first treatment.


Assuntos
Leiomiossarcoma , Neoplasias Uterinas , Adulto , Idoso , China/epidemiologia , Feminino , Humanos , Histerectomia , Leiomiossarcoma/diagnóstico , Leiomiossarcoma/patologia , Leiomiossarcoma/cirurgia , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Reoperação , Estudos Retrospectivos , Neoplasias Uterinas/diagnóstico , Neoplasias Uterinas/patologia , Neoplasias Uterinas/cirurgia , Adulto Jovem
5.
Biochem Biophys Res Commun ; 570: 117-124, 2021 09 17.
Artigo em Inglês | MEDLINE | ID: mdl-34280614

RESUMO

Kinesin Family Member 15 (KIF15) is a plus end-directed microtubule motor, which exerts complex regulations in cancer biology. This study aimed to explore the functional role of KIF15 in leiomyosarcoma (LMS). Bioinformatic analysis was carried out using data from The Cancer Genome Atlas (TCGA)-Sarcoma (SARC). LMS cell lines SK-UT-1 and SK-LMS-1 were used as in vitro cell models. Results showed that LMS patients with high KIF15 expression had significantly worse survival than the low KIF15 expression counterparts. KIF15 knockdown slowed, while KIF15 overexpression increased the proliferation of SK-UT-1 and SK-LMS-1 cells. Co-IP assay confirmed mutual interaction between endogenous KIF15 and DEK (encoded by DEK proto-oncogene). KIF15 knockdown facilitated DEK degradation, while KIF15 overexpression slowed DEK degradation. In ubiquitination assay, a significant increase in DEK polyubiquitylation was observed when KIF15 expression was suppressed. USP15 physically interacted with both DEK and KIF15 in the cells. USP15 knockdown decreased DEK protein stability and canceled KIF15-mediated DEK stabilization. USP15 overexpression enhanced DEK stability, the effect of which was impaired by KIF15 knockdown. USP15 overexpression reduced DEK polyubiquitination. USP15 knockdown increased DEK polyubiquitination and canceled the effect of KIF15 overexpression on reducing DEK polyubiquitination. DEK overexpression enhanced the proliferation of SK-UT-1 and SK-LMS-1 cells. DEK knockdown decreased cell proliferation and canceled the effect of KIF15 overexpression on cell proliferation. In conclusion, this study revealed a novel mechanism that KIF15 enhances LMS cell proliferation via preventing DEK protein from degradation by increasing USP15 mediated deubiquitylation.


Assuntos
Proteínas Cromossômicas não Histona/metabolismo , Regulação Neoplásica da Expressão Gênica , Cinesina/genética , Leiomiossarcoma/genética , Leiomiossarcoma/patologia , Proteínas Oncogênicas/metabolismo , Proteínas de Ligação a Poli-ADP-Ribose/metabolismo , Proteases Específicas de Ubiquitina/metabolismo , Ubiquitinação , Regulação para Cima/genética , Linhagem Celular Tumoral , Proliferação de Células/genética , Humanos , Cinesina/metabolismo , Complexo de Endopeptidases do Proteassoma/metabolismo , Ligação Proteica , Estabilidade Proteica , Proteólise
6.
Afr J Reprod Health ; 25(1): 161-168, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34077121

RESUMO

Primary vaginal leiomyosarcoma is a rare gynecological malignancy. The clinical presentation is a benign looking well circumscribed mobile mass which might however occasionally present with distant metastases. Post treatment recurrence is common, and the clinical course is often unpredictable. Primary surgical management plus radiotherapy is the commonly practiced treatment of choice. We report a case of primary vaginal epithelioid leiomyosarcoma that recurred twice after local surgical resection. The patient subsequently underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy for a recurrent vaginal leiomyosarcoma. Histopathology of the vaginal mass revealed an epithelioid leiomyosarcoma of the vagina. She also received a course of adjuvant radiotherapy. The patient was free of recurrence at 3 year follow up. Vaginal mass must be evaluated with a high index of suspicion for malignancy. Local surgical resection alone is insufficient for primary vaginal leiomyosarcoma. Surgical resection with adjuvant radiotherapy offers better treatment outcomes with decreased risk of recurrence. Empirical oophorectomy in patients with completed family size might confer additional benefit in preventing disease recurrence in resource limited settings where testing for estrogen and progesterone receptor status of the tumour is unavailable. Due to the unpredictable course of the disease, lifelong patient follow-up is critical for better outcomes.


Assuntos
Histerectomia/métodos , Leiomioma/cirurgia , Leiomiossarcoma/terapia , Radioterapia Adjuvante/efeitos adversos , Salpingo-Ooforectomia/métodos , Neoplasias Vaginais/cirurgia , Botsuana , Feminino , Humanos , Leiomioma/patologia , Leiomiossarcoma/patologia , Leiomiossarcoma/secundário , Leiomiossarcoma/cirurgia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Resultado do Tratamento , Neoplasias Uterinas/patologia , Neoplasias Uterinas/terapia , Vagina/patologia , Vagina/cirurgia , Neoplasias Vaginais/patologia
7.
Anticancer Res ; 41(6): 3001-3010, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34083291

RESUMO

BACKGROUND/AIM: During pregnancy, uterine leiomyomas may cause problems and treatment typically entails uterine conservation. However, for cases of leiomyomas larger than a particular size with some clinical symptoms, enucleation should be performed. In clinical practice, the importance of postpartum follow-up of pregnancies with uterine fibroids must be established. PATIENTS AND METHODS: A 47-year-old female visited an obstetrics and gynecology clinic with a primary complaint of irregular bleeding. We suspected an 8.4×6.6 cm myoma uteri and recommended immediate surgery. During the next visit, a pregnancy test was positive and the patient requested a follow-up for her myoma uteri diagnosis. Because of a breech presentation, we performed an elective cesarean section (CS) at 38 weeks and 1 day. The patient's myoma uteri was stable throughout the pregnancy, and after delivery, we continued to follow her up as an outpatient. RESULTS: Two years after the CS, the myoma uterus was 6 cm in size, and 6 months later, it had increased to 10 cm. Magnetic resonance imaging (MRI) supported a diagnosis of uterine leiomyosarcoma and she underwent surgery. Ultimately, she was pathologically diagnosed with uterine leiomyoma, uterine leiomyoma with bizarre nuclei, and uterine leiomyosarcoma following examination of the excised tissue by using molecular pathological examination with anti-cyclin E antibody and anti-Ki-67 antibody. CONCLUSION: Notably, this case demonstrated the usefulness of cyclin E and Ki-67 as biomarkers for the malignancy of uterine mesenchymal tumors. Presently, she is being monitored for tumor recurrence and metastases on a quarterly basis. In order to detect the rapid increase in uterine mesenchymal tumor, regular follow-up after birth is important.


Assuntos
Cesárea , Leiomioma/patologia , Leiomiossarcoma/patologia , Neoplasias Uterinas/patologia , Progressão da Doença , Feminino , Seguimentos , Humanos , Leiomioma/diagnóstico por imagem , Leiomiossarcoma/diagnóstico por imagem , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Gravidez , Neoplasias Uterinas/diagnóstico por imagem
8.
Am J Surg Pathol ; 45(9): 1179-1189, 2021 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-34074809

RESUMO

Frozen sections of uterine smooth muscle tumors are infrequently required, and related diagnostic difficulties are seldom discussed. We analyzed the clinicopathologic features of 112 frozen sections of uterine smooth muscle tumors and determined the accuracy, reasons for deferrals, and causes of interpretational errors. Most patients (median age, 45 y) presented with pelvic mass symptoms (53%). The main reasons for a frozen section examination were an abnormal gross appearance including loss of the usual whorled pattern of leiomyoma (36 cases, 32.1%), and intraoperative discovery of an abnormal growth pattern and extrauterine extension of a uterine tumor (28 cases, 25%). There were 9 leiomyosarcomas and 103 leiomyomas, including 18 benign histologic variants. An accurate diagnosis of malignancy was achieved in all leiomyosarcomas, with the exception of a myxoid leiomyosarcoma. In 99 cases (88%), the frozen section diagnosis concurred with the permanent section diagnosis (false positives, 0.9%; false negatives, 0%). Misinterpretation of stromal hyalinization as tumor cell necrosis in a leiomyoma with amianthoid-like fibers was a major discrepancy. Two minor discrepancies did not lead to a change in management. The diagnosis was deferred in 10 cases (8.9%) because of stromal alterations, unusual cellular morphology, uncertain type of necrosis, and abnormal growth patterns. Thus, although various stromal and cellular alterations can cause diagnostic uncertainty, leading to deferrals, frozen section diagnosis of uterine smooth muscle tumors has a high accuracy rate. While a definitive frozen section diagnosis of malignancy may be made when there is unequivocal atypia, indisputable mitotic figures, and tumor cell necrosis, it is important to remember that nonmyogenic mesenchymal tumors may also mimic uterine smooth muscle tumors. In a frozen section setting, it would be sufficient to issue a diagnosis of "malignant mesenchymal tumor." For tumors that do not meet the criteria for malignancy, issuing a frozen section diagnosis of "atypical mesenchymal tumor and defer the histologic subtyping to the permanent sections" is appropriate.


Assuntos
Secções Congeladas/métodos , Leiomioma/diagnóstico , Leiomiossarcoma/diagnóstico , Neoplasias Uterinas/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Feminino , Humanos , Período Intraoperatório , Leiomioma/patologia , Leiomiossarcoma/patologia , Pessoa de Meia-Idade , Tumor de Músculo Liso/diagnóstico , Tumor de Músculo Liso/patologia , Neoplasias Uterinas/patologia
9.
Chest ; 159(5): e313-e317, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33965155

RESUMO

CASE PRESENTATION: A 26-year-old man presented with a 2-week history of productive cough and a 1-year history of effort-related dyspnea. His medical history was significant for hay fever and exertion-triggered asthma. He was not taking medicines regularly but was using inhaled salbutamol as needed. He was an ex-smoker, with a previous history of 2-pack years.


Assuntos
Leiomiossarcoma/diagnóstico , Leiomiossarcoma/cirurgia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/cirurgia , Adulto , Broncoscopia , Diagnóstico Diferencial , Diagnóstico por Imagem , Humanos , Leiomiossarcoma/patologia , Neoplasias Pulmonares/patologia , Excisão de Linfonodo , Masculino , Pneumonectomia , Fumantes
10.
Sci Rep ; 11(1): 9765, 2021 05 07.
Artigo em Inglês | MEDLINE | ID: mdl-33963205

RESUMO

Soft tissue sarcoma represents about 1% of all adult cancers. Occurrence of multiple sarcomas in a same individual cannot be fortuitous. A 72-year-old patient had between 2007 and 2016 a glomangiopericytal tumor of the right forearm and a succession of sarcomas of the extremities: a leiomyosarcoma of the left buttock, a myxofibrosarcoma (MFS) of the right forearm, a MFS of the left scapula, a left latero-thoracic MFS and two undifferentiated sarcomas on the left forearm. Pathological examination of the six locations was not in favor of disease with local/distant recurrences but could not confirm different diseases. An extensive molecular analysis including DNA-array, RNA-sequencing and DNA-Sanger-sequencing, was thus performed to determine the link between them. The genomic profile of the glomangiopericytal tumor and the six sarcomas revealed that five sarcomas were different diseases and one was the local recurrence of the glomangiopericytal tumor. While the chromosomal alterations in the six tumors were different, a common somatic CDKN2A/CDKN2B deletion was identified. RNA-sequencing of five tumors identified mutations in GLT8D1, GATAD2A and SLC25A39 in all samples. The germline origin of these mutations was confirmed by Sanger-sequencing. Innovative molecular analysis methods have made possible a better understanding of the complex tumorigenesis of multiple sarcomas.


Assuntos
Fibrossarcoma/genética , Mutação em Linhagem Germinativa , Glicosiltransferases/genética , Leiomiossarcoma/genética , Proteínas de Transporte da Membrana Mitocondrial/genética , Proteínas de Neoplasias/genética , Segunda Neoplasia Primária/genética , Proteínas Repressoras/genética , Neoplasias de Tecidos Moles/genética , Idoso , Fibrossarcoma/patologia , Humanos , Leiomiossarcoma/patologia , Masculino , Segunda Neoplasia Primária/patologia , Neoplasias de Tecidos Moles/patologia
11.
Oxid Med Cell Longev ; 2021: 9999529, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34007412

RESUMO

Myxosarcomas are rare malignant tumors of soft connective tissues, classified into various subtypes, including myxoid liposarcoma, myxoid chondrosarcoma, and myxoid leiomyosarcoma. In this study, we proposed to study the demographic, tumor characteristics, and overall survival rate and compared the treatment modalities between these cancers. Patient data collected based on locoregional metastasis presentation of the abovementioned tumors with a cutoff study of survival duration up to 10 years were obtained from the SEER database during 1975-2016. Our results indicated that elderly patients and females were more in locoregional myxoid leiomyosarcoma than myxoid liposarcoma and myxoid chondrosarcoma with locoregional metastasis. The white race represented the most patients who suffered from these cancers than other races. The heart is the primary site for the abovementioned cancers, in addition to the female genitals to the myxoid leiomyosarcoma. Myxoid liposarcoma and myxoid chondrosarcoma patients with locoregional metastasis were suffering from grade II, while locoregional myxoid leiomyosarcoma patients with blank grading were due to missed data. Surgery was the most common treatment modality in this study compared with radiotherapy and chemotherapy. Kaplan-Meier analysis showed a significant difference in survival time between the three subtypes by using histology, and myxoid leiomyosarcoma showed prolonged survival than others. Elderly, female, white, unknown grade, surgery, no radiation, and no chemotherapy variables were independent factors associated with overall survival among these cancers. Multivariate analysis also showed significant differences in overall survival between the three tumors by histology, and myxoid leiomyosarcoma was with a better prognosis than others. Multivariate analysis of locoregional myxoid leiomyosarcoma showed the statistical significance of black race, grade, and radiotherapy, indicating them as independent prognostic factors of locoregional myxoid leiomyosarcoma. We conclude that surgery was the primary treatment modality against these cancers than radiotherapy and chemotherapy. And the locoregional myxoid leiomyosarcomas showed a better prognosis and higher survival rate than locoregional myxoid liposarcoma and locoregional myxoid chondrosarcoma.


Assuntos
Leiomiossarcoma/mortalidade , Leiomiossarcoma/cirurgia , Mixossarcoma/mortalidade , Mixossarcoma/cirurgia , Feminino , Humanos , Leiomiossarcoma/patologia , Pessoa de Meia-Idade , Mixossarcoma/patologia , Taxa de Sobrevida , Estados Unidos
12.
Clin Orthop Relat Res ; 479(5): 1134-1143, 2021 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-33861216

RESUMO

BACKGROUND: Heterotopic ossification (HO) is common after total joint arthroplasty and usually does not cause diagnostic problems. However, the occurrence of HO after oncologic prostheses implantation can be troublesome as it may mimic a locally recurrent tumor. Because this distinction could have a profound impact on the surgeon and patient, it is important to distinguish the two entities; to our knowledge, no study has evaluated this after oncologic endoprosthetic reconstruction around the knee after tumor resection. QUESTIONS/PURPOSES: (1) How common is the occurrence of HO compared with local recurrence (LR) after resection of bone sarcoma and the use of an oncologic knee prosthesis? (2) Are there any factors associated with the development of HO after limb salvage procedures with an endoprosthesis? (3) What features allow the surgeon to differentiate HO from a locally recurrent tumor in this setting? METHODS: Between 2002 and 2018, we performed 409 resections of primary bone tumors followed by reconstructions with oncologic endoprostheses. Of these, 17% (71 of 409) died before 2 years and did not have HO at that time, 2% (8 of 409) were lost to follow-up before 2 years, and another 2% (10 of 409) did not have radiographs available at a minimum of 2 years after surgery (and had not developed HO before then), and so could not be analyzed, leaving 320 patients for analysis in this retrospective study. Forty-two patients were excluded; 2% (5 of 320) for a history of failed allograft reconstruction, 3% (8 of 320) for pathologic fracture at presentation, 2% (6 of 320) for inadequate or complicated biopsy, 1% (2 of 320) for stem fractures, 2% (7 of 320) for stem loosening, and 4% (14 of 320) for extracortical bone bridging, leaving 278 patients for final evaluation. Two observers analyzed AP and lateral radiographs for signs of HO at a mean follow-up of 63 ± 33 months after surgery. We defined HO as extraskeletal bone formation in soft tissues. The primary study endpoint was survivorship free from HO, as ascertained by a competing-risks estimator. To identify factors associated with HO appearance, the demographic, radiographic, clinical, pathologic, and surgical characteristics were compared between patients with HO and those who had no lesion. Characteristic features were also compared between patients with HO and those with LR to help their differentiation. Univariate analysis was used for all statistical evaluations. RESULTS: HO developed in 8% (21 of 278) of patients in whom oncologic knee prosthesis was implanted. LR developed in 10% (28 of 278) of the patients. According to survivorship estimates, the HO-free survival rate was not different from the LR-free survival rate at 2 years after oncologic knee reconstruction (76 ± 5% [95% CI 63 to 87] versus 74 ± 5% [95% CI 62 to 88]; p = 0.19). History of infection was more common in patients with HO than in patients with no lesion (19% [4 of 21] versus 5% [12 of 229], Odds ratio [OR] 6 [95% CI 2 to 17]; p < 0.001). The male sex was more common in the HO group as well (76% [16 of 21] versus 55% [128 of 229], OR 2 [95% CI 1 to 5]; p = 0.03). The Modular Universal Tumor and Revision System prosthesis was more frequently used in patients with HO (67% [14 of 21]) compared to those with no lesions (40% [92 of 229]; OR 2 [95% CI 1 to 5]; p = 0.02). The lesion border in radiographs was ill-defined in 19% (4 of 21) of patients with HO and 100% (28 of 28) of patients with LR (OR 8 [95% CI 3 to 20]; p < 0.001). The median time to the appearance of HO was shorter than the time to LR (8 months [3 to 13] versus 16 months [11 to 21], [95% CI 10 to 13]; p < 0.001). Pain at presentation was more frequent in patients with LR than in those with HO (86% [24 of 28] versus 14% [3 of 21], OR 36 [95% CI 7 to 181]; p < 0.001). CONCLUSION: HO may occur after the use of oncologic knee prostheses for reconstruction after tumor resection. In most patients, HO could be differentiated from local recurrence through identifying a well-defined border on radiographs. Otherwise, factors such as an earlier time of presentation and absence of pain could suggest an HO, rather than an LR. LEVEL OF EVIDENCE: Level III, therapeutic study.


Assuntos
Artroplastia do Joelho/instrumentação , Neoplasias Ósseas/cirurgia , Condrossarcoma/cirurgia , Histiocitoma Fibroso Maligno/cirurgia , Articulação do Joelho/cirurgia , Prótese do Joelho , Leiomiossarcoma/cirurgia , Recidiva Local de Neoplasia , Ossificação Heterotópica/diagnóstico por imagem , Osteossarcoma/cirurgia , Osteotomia , Adolescente , Adulto , Artroplastia do Joelho/efeitos adversos , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/patologia , Diagnóstico Diferencial , Feminino , Histiocitoma Fibroso Maligno/diagnóstico por imagem , Histiocitoma Fibroso Maligno/patologia , Humanos , Articulação do Joelho/diagnóstico por imagem , Articulação do Joelho/fisiopatologia , Leiomiossarcoma/diagnóstico por imagem , Leiomiossarcoma/patologia , Masculino , Ossificação Heterotópica/etiologia , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/patologia , Osteotomia/efeitos adversos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
13.
Magy Seb ; 74(1): 22-26, 2021 Mar 16.
Artigo em Inglês | MEDLINE | ID: mdl-33729993

RESUMO

Összefoglaló. A vena cava inferior leiomyosarcomája a vena tunica mediájából kiinduló igen ritka rosszindulatú daganat. Tünetei és radiomorfológiai jelei nem mindig juttatnak pontos kórisméhez, szövettani mintavétele pedig elhelyezkedése miatt veszélyes lehet. Diagnózisa ezért sokszor jelent kihívást a klinikusok számára. Kezelése elsosorban sebészi, amelyet együtt vagy monoterápiában alkalmazott radioterápia és kemoterápia egészít ki. Esetünkben egy 74 éves nobeteg tünetmentes, a v. cava inferior jobb v. renalis fölötti részének jobb mellékvesével összefüggo tumorát találtuk. Endokrinológiai kivizsgálása során a szérum kortizol, adrenalin, noradrenalin, adenocorticotrop hormon (ACTH), teljes és szabad tesztoszteron, dehidroepiandroszteron-szulfát (DHEA-S), nemi hormon kötofehérje (sex hormone binding globulin, SHBG) normál tartományban volt, a tumor hormonszekréciót nem mutatott. A tumor sebészi eltávolítása mellett döntöttünk. Preoperatív biopsziát annak kockázatai miatt nem végeztünk. A mutét során a v. cava inferior daganatát találtuk, a tumor eltávolítását végeztük a vena falának resectiójával, a jobb mellékvese eltávolításával és a jobb v. renalis neoimplantatiójával. A jobb feltárás érdekében a mutét elején cholecystectómiát is végeztünk. A mutét után szövodmény nem jelentkezett. A beteg adjuváns radioterápiában részesült. A tumor korai diagnózisának és en bloc resectiójának köszönhetoen jelentosen növeltük a beteg gyógyulási esélyeit, az elvégzett radioterápiával pedig csökkentettük a recidíva valószínuségét. Summary. Leiomyosacroma of the inferior vena cava is an extremely rare malignancy originating from the tunica media of the venous wall. Its symptoms and radiomorphological signs do not always lead to an accurate diagnosis. Histological sampling can be dangerous due to its location. Therefore the diagnosis is often a challenge for clinicians. Its treatment is primarily surgical, supplemented by radiotherapy and chemotherapy applied together or in monotherapy. In our case, an asymptomatic 74-year-old female patient was diagnosed with a tumor of the inferior caval vein located just above the right renal vein and involving the right adrenal gland. As serum cortisol, epinephrine, norepinephrine, adrenocorticotropic hormone (ACTH), total and free testosterone, dehydroepiandrosterone sulfate (DHEA-S), sex hormone binding globulin (SHBG) was at normal level, the tumor showed no hormone secretion. Primary surgical resection was planned. Preoperative biopsy was not performed due to its high risk of complications. During operation, tumor resection was performed by resection of the venous wall, removal of the right adrenal gland and neoimplantation of the right renal vein. For better exposure, cholecystectomy was also performed at the beginning of surgery. There were no postoperative complications. The patient received adjuvant radiation therapy. Thanks to the early diagnosis of the tumor and the complete resection, we significantly increased the patient's chances of total recovery. With the radiotherapy performed, we reduced the likelihood of tumor recurrence.


Assuntos
Leiomiossarcoma/cirurgia , Neoplasias Vasculares/terapia , Veia Cava Inferior/cirurgia , Adrenalectomia , Idoso , Colecistectomia , Feminino , Humanos , Leiomiossarcoma/patologia , Recidiva Local de Neoplasia , Veias Renais/patologia , Veias Renais/cirurgia , Resultado do Tratamento , Neoplasias Vasculares/patologia , Veia Cava Inferior/patologia
14.
J Surg Oncol ; 123(7): 1618-1623, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33650695

RESUMO

BACKGROUND AND OBJECTIVES: Caval leiomyosarcomas (cLMS) are rare soft tissue sarcomas historically associated with high recurrence rates and poor prognosis. While radical resection remains the mainstay of therapy for cLMS, new systemic therapies have presented opportunities for multimodality treatment. We examined the clinical outcomes of patients with cLMS treated with modern, multimodality approaches, and compared their outcomes to those of patients with noncaval retroperitoneal LMS (ncLMS). METHODS: A retrospective, single-institution review identified all patients diagnosed with primary retroperitoneal LMS from 2012 to 2018. Radiographic and pathologic review distinguished patients with cLMS and ncLMS. Standard clinicopathologic variables and response to chemotherapy (when applicable) were analyzed. Primary endpoints were overall (OS) and progression-free survival (PFS). RESULTS: Eleven patients with cLMS were identified. Median tumor size was 7.5 cm (IQR, 5.0-14.3 cm); all patients had Stage II/III disease. Seven patients received neoadjuvant chemotherapy. Nine cLMS patients underwent R0/R1 resection; two did not complete resection. Six patients received adjuvant systemic therapy. Twenty patients with ncLMS were treated during the same period. No statistical intergroup differences were noted in tumor size, pathologic grade, stage, or resection margin status. Patients with ncLMS were less likely to receive neoadjuvant (10% vs. 64%) and adjuvant chemotherapy (30% vs. 55%). Two-year OS (81% vs. 78%; p = NS) and PFS (55% vs. 46%; p = NS) were comparable between cLMS and ncLMS patients. CONCLUSIONS: Multimodality treatment with systemic therapy and aggressive surgical resection may achieve equivalent survival outcomes for patients with cLMS versus similar ncLMS. We recommend that all patients with cLMS be evaluated for multidisciplinary treatment. Genomic and proteomic expression profiling may identify novel or targetable mutations.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leiomiossarcoma/tratamento farmacológico , Leiomiossarcoma/cirurgia , Neoplasias Retroperitoneais/tratamento farmacológico , Neoplasias Retroperitoneais/cirurgia , Neoplasias Vasculares/tratamento farmacológico , Neoplasias Vasculares/cirurgia , Veia Cava Inferior/patologia , Anticorpos Monoclonais/administração & dosagem , Estudos de Coortes , Dacarbazina/administração & dosagem , Doxorrubicina/administração & dosagem , Feminino , Humanos , Ifosfamida/administração & dosagem , Leiomiossarcoma/genética , Leiomiossarcoma/patologia , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante , Estadiamento de Neoplasias , Neoplasias Retroperitoneais/genética , Neoplasias Retroperitoneais/patologia , Estudos Retrospectivos , Neoplasias Vasculares/genética , Neoplasias Vasculares/patologia , Veia Cava Inferior/cirurgia
15.
Indian J Cancer ; 58(2): 267-272, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33753608

RESUMO

We herein describe a case of primary leiomyosarcoma of descending mesocolon mesentery in a pregnant woman. A 31-year-old woman was referred to our clinic for the presence of a suspicious mass (solid heterogenous lesion with lobulated margins) detected during routine obstetric ultrasonography (USG), growing throughout her term. Imaging in her third trimester showed a considerable increase in the size of the mass and was suspected to be malignancy of uterine origin. Tru-cut biopsy performed post-partum indicated leiomyosarcoma. She underwent neoadjuvant chemotherapy with six cycles of dacarbazine and doxorubicin with partial response. Subsequently, she underwent surgery, and the tumor was found to be present in sigmoid colon mesentery extending in retroperitoneum involving 5 cm of ureter. The mass was resected along with part of the colon and ureter that was involved by disease. Patient had uneventful recovery post-surgery. Considering moderate response to chemotherapy and discussion in tumor board, she was not given adjuvant chemotherapy. At follow-up of 15 months, the patient is disease-free with a normal, healthy baby.


Assuntos
Leiomiossarcoma/patologia , Mesentério/patologia , Terapia Neoadjuvante/métodos , Complicações Neoplásicas na Gravidez/patologia , Gestantes , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Quimioterapia Adjuvante , Feminino , Humanos , Leiomiossarcoma/tratamento farmacológico , Mesentério/efeitos dos fármacos , Gravidez , Complicações Neoplásicas na Gravidez/tratamento farmacológico
16.
J Surg Oncol ; 123(5): 1274-1283, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33524203

RESUMO

BACKGROUNDS AND OBJECTIVES: This investigation sought to describe the outcomes of primary leiomyosarcoma of bone (PLB) compared to soft tissue leiomyosarcoma (SLMS). METHODS: This was a review of the Surveillance, Epidemiology, and End Results database from 1975 to 2016. Kaplan-Meier methods were used to estimate disease-specific survival (DSS), and a Cox regression model was used to identify prognostic factors. RESULTS: Of the 7502 identifiable cases, 1% (n = 74) were PLB and 99% (n = 7428) were SLMS. Survival was the same between PLB and SLMS (p = .209). On multivariable analysis for high-grade SLMS, radiation (neoadjuvant: hazard ratio [HR], 0.56; 95% confidence interval [CI], 0.4-0.8; p = .003; adjuvant: HR, 0.75; 95% CI, 0.6-0.9; p = .008) and surgery (procedure specific) improved DSS. For PLB, wide resection/limb salvage (HR, 0.40; 95% CI, 0.3-0.5; p = .018) and amputation (HR, 0.69; 95% CI, 0.5-0.9; p < .001) were positive prognostic factors. Neither radiation nor chemotherapy were prognostic factors for survival in PLB. CONCLUSIONS: For SLMS, radiation portends a survival advantage. For PLB, however, neither chemotherapy nor radiation were significant prognostic factors, which suggests the optimal treatment for PLB, similar to other primary soft tissue sarcomas originating in bone, remains an unmet medical need.


Assuntos
Neoplasias Ósseas/mortalidade , Leiomiossarcoma/mortalidade , Neoplasias de Tecidos Moles/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/patologia , Neoplasias Ósseas/terapia , Criança , Pré-Escolar , Estudos de Coortes , Terapia Combinada , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Leiomiossarcoma/patologia , Leiomiossarcoma/terapia , Pessoa de Meia-Idade , Prognóstico , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/terapia , Taxa de Sobrevida , Adulto Jovem
17.
Int J Mol Sci ; 22(4)2021 Feb 04.
Artigo em Inglês | MEDLINE | ID: mdl-33557274

RESUMO

Uterine smooth muscle tumors of uncertain malignant potential (STUMPs) represent a heterogeneous group of tumors that cannot be histologically diagnosed as unequivocally benign or malignant. For this reason, many authors are working to obtain a better definition of diagnostic and prognostic criteria. In this work, we analyzed the genomic and epigenomic profile of uterine smooth muscle tumors (USMTs) in order to find similarities and differences between STUMPs, leiomyosarcomas (LMSs) and leiomyomas (LMs), and possibly identify prognostic factors in this group of tumors. Array-CGH data on 23 USMTs demonstrated the presence of a more similar genomic profile between STUMPs and LMSs. Some genes, such as PRKDC and PUM2, with a potential prognostic value, were never previously associated with STUMP. The methylation data appears to be very promising, especially with regards to the divergent profile found in the sample that relapsed, characterized by an overall CGI hypomethylation. Finally, the Gene Ontology analysis highlighted some cancer genes that could play a pivotal role in the unexpected aggressive behavior that can be found in some of these tumors. These genes could prove to be prognostic markers in the future.


Assuntos
Biomarcadores Tumorais/genética , Epigenômica , Regulação Neoplásica da Expressão Gênica , Leiomioma/patologia , Leiomiossarcoma/patologia , Tumor de Músculo Liso/patologia , Neoplasias Uterinas/patologia , Adulto , Idoso , Estudos de Casos e Controles , Metilação de DNA , Feminino , Seguimentos , Genômica , Humanos , Leiomioma/genética , Leiomiossarcoma/genética , Masculino , Pessoa de Meia-Idade , Prognóstico , Tumor de Músculo Liso/genética , Neoplasias Uterinas/genética
18.
J Surg Oncol ; 123(4): 1081-1087, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33444466

RESUMO

BACKGROUND: The role of 18 F-fluorodeoxyglucose positron emission tomography/computed tomography (18 F-FDG PET/CT) in the evaluation of retroperitoneal sarcomas is poorly defined. We evaluated the correlation of maximum standardized uptake value (SUVmax) with pathologic tumor grade in the surgical specimen of primary retroperitoneal dedifferentiated liposarcoma (DDLPS) and leiomyosarcoma (LMS). METHODS: Patients with the above histological subtypes in three participating institutions with preoperative 18 F-FDG PET/CT scan and histopathological specimen available for review were included. The association between SUVmax and pathological grade was assessed. Correlation between SUVmax and relapse-free survival (RFS) and overall survival (OS) were also studied. RESULTS: Of the total 58 patients, final pathological subtype was DDLPS in 44 (75.9%) patients and LMS in 14 (24.1%) patients. The mean SUVmax was 8.7 with a median 7.1 (range, 2.2-33.9). The tumors were graded I, II, III in 6 (10.3%), 35 (60.3%), and 17 (29.3%) patients, respectively. There was an association of higher histological grade with higher SUVmax (rs = 0.40, p = .002). Increasing SUVmax was associated with worse RFS (p = .003) and OS (p = .003). CONCLUSION: There is a correlation between SUVmax and pathologic tumor grade; increasing SUVmax was associated with worse OS and RFS, providing a preoperative noninvasive surrogate marker of tumor grade and biological behavior.


Assuntos
Leiomiossarcoma/mortalidade , Lipossarcoma/mortalidade , Recidiva Local de Neoplasia/mortalidade , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Neoplasias Retroperitoneais/mortalidade , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Fluordesoxiglucose F18/metabolismo , Seguimentos , Humanos , Leiomiossarcoma/diagnóstico por imagem , Leiomiossarcoma/patologia , Leiomiossarcoma/cirurgia , Lipossarcoma/diagnóstico por imagem , Lipossarcoma/patologia , Lipossarcoma/cirurgia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/diagnóstico por imagem , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/cirurgia , Prognóstico , Compostos Radiofarmacêuticos/metabolismo , Neoplasias Retroperitoneais/diagnóstico por imagem , Neoplasias Retroperitoneais/patologia , Neoplasias Retroperitoneais/cirurgia , Estudos Retrospectivos , Taxa de Sobrevida
19.
BMJ Case Rep ; 14(1)2021 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-33495185

RESUMO

Appendiceal neoplasms are rare, occurring in <1.4% of all appendicectomy specimens. Carcinoid tumours and adenocarcinomas comprise the majority of cases, however, lymphomas or sarcomas may also arise within the appendix. Appendiceal leiomyosarcomas are rare and to date, there remains a relative dearth of cases reported in the literature. Leiomyosarcomas are derived from the smooth muscle cells or mesenchymal stem cells committed to this line of differentiation. However, their pathogenesis and underlying genetic mechanism remains to be fully elucidated. Unbalanced karyotypic defects are the only shared features observed across different leiomyosarcoma subtypes. Children with AIDS have a higher incidence compared with adults, where the main pathology in individuals with HIV is Kaposi's sarcoma and B-cell lymphoma. Although surgical excision with clear margins remains the treatment of choice, a good response to treatment with gemcitabine, docetaxel and trabectedin has been observed. The authors present the case of a 23-year-old female presenting to the emergency department with acute appendicitis. She underwent a laparoscopic converted to an open appendectomy. Her operation was complicated by a pelvic collection requiring percutaneous drainage and an ileus. Histopathological examination confirmed the diagnosis of a leiomyosarcoma, a rare mesenchymal tumour presenting in individuals with immune suppression. HIV serology was positive and she commenced anti-retroviral therapy. She remains under review in the Department of HIV Medicine.


Assuntos
Neoplasias do Apêndice/diagnóstico , Apendicite/diagnóstico , Infecções por HIV/diagnóstico , Leiomiossarcoma/diagnóstico , Injúria Renal Aguda , Apendicectomia , Neoplasias do Apêndice/imunologia , Neoplasias do Apêndice/patologia , Neoplasias do Apêndice/cirurgia , Diagnóstico Diferencial , Feminino , Infecções por HIV/imunologia , Humanos , Hipopotassemia , Íleus , Hospedeiro Imunocomprometido , Leiomiossarcoma/imunologia , Leiomiossarcoma/patologia , Leiomiossarcoma/cirurgia , Complicações Pós-Operatórias , Infecção da Ferida Cirúrgica , Adulto Jovem
20.
Am J Dermatopathol ; 43(6): 446-449, 2021 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-33464746

RESUMO

ABSTRACT: Leiomyosarcoma is a common sarcoma of both organs and soft tissues; however, large intradermal tumors are extremely rare. Presented is a pleomorphic leiomyosarcoma in a 64-year-old man, initially considered to be a ruptured epidermal inclusion cyst. The patient had a mildly tender, enlarging soft-tissue mass with a central pore on his right upper back. Incomplete extirpation showed a 5 × 5 cm heterogeneous, predominantly pleomorphic sarcoma with areas of fascicular and storiform spindled cells infiltrating the subcutaneous soft tissue to the underlying fascia and extending upward into the middle and upper dermis with prominent extension into pilosebaceous units. There were small foci with myxoid stroma and large areas of necrosis. CD31 demonstrated thin-walled curvilinear vessels throughout the tumor. The first desmin immunohistochemical stain near areas with myxoid stroma was negative but smooth muscle actin positive. However, desmin positivity was strong and diffuse in the spindled and more pleomorphic areas on 2 additional tissue sections. No rhabdomyoblasts or striated muscle fibers were seen. A diagnosis of pleomorphic leiomyosarcoma was rendered. This case highlights a unique clinical and histological presentation of a leiomyosarcoma initially mistaken to be a ruptured epidermal inclusion cyst, and the need to sometimes apply ancillary immunohistochemical studies to sections from more than one tissue block to accurately differentiate heterogeneous sarcomas with similar histologic features.


Assuntos
Leiomiossarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Diagnóstico Diferencial , Cisto Epidérmico/patologia , Humanos , Masculino , Pessoa de Meia-Idade
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