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1.
J Cancer Res Clin Oncol ; 147(4): 1137-1144, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33550433

RESUMO

BACKGROUND: Neoplasms of the retroperitoneum that contain a major fat component may represent either benign entities, such as lipomas or angiomyolipomas, or malignancy such as liposarcoma. Distinguishing these diagnoses has important implications for management. While liposarcomas often stain positively for MDM2 and CDK4 proteins, absence of these markers can lead to diagnostic and management challenges. METHODS: We examined three cases in our institution of fat-containing masses of the retroperitoneum that lacked MDM2 and CDK4 markers to highlight the challenges in diagnosing and managing these cases. A thorough review of the literature examining radiologic and histologic features that can be used to determine that diagnosis was conducted and summarized. RESULTS: The three cases we present represent the three main diagnostic entities that can be found in among fatty tumors of the retroperitoneum: lipoma, angiomyolipoma, and liposarcoma. While radiologic features and analysis of histology helped to inform management, these cases in conjunction with the literature also illustrate the limitations of the diagnostic work up and importance also factoring the biologic behavior of the tumor in its management. CONCLUSION: Fat-containing tumors of the retroperitoneum that do not stain for MDM2 or CDK4 can pose a diagnostic challenge. Assessing radiologic and pathologic features in conjunction with the biologic behavior of these tumors should inform their management.


Assuntos
Quinase 4 Dependente de Ciclina/metabolismo , Lipoma/diagnóstico , Lipoma/terapia , Proteínas Proto-Oncogênicas c-mdm2/metabolismo , Neoplasias Retroperitoneais/diagnóstico , Neoplasias Retroperitoneais/terapia , Animais , Gerenciamento Clínico , Humanos , Lipoma/metabolismo , Neoplasias Retroperitoneais/metabolismo
2.
Diagn Pathol ; 16(1): 2, 2021 Jan 09.
Artigo em Inglês | MEDLINE | ID: mdl-33419470

RESUMO

BACKGROUND: IL4Rα and IL13Rα1 are constituents of the type II IL4 receptor. Recently, IL4Rα and IL13Rα1 were reported to have roles in cancer progression and suggested as potential prognostic markers. However, studies on IL4Rα and IL13Rα1 in soft-tissue sarcomas have been limited. METHODS: This study investigated the immunohistochemical expression of IL4Rα and IL13Rα1 in 89 soft-tissue sarcomas of the extremities, superficial trunk, and retroperitoneum. Immunohistochemical staining for IL4Rα and IL13Rα1 were scored according to a combination of staining intensity and staining area in tissue microarray samples. Positivity for the immunohistochemical expression of IL4Rα and IL13Rα1 were determined using receiver operating curve analysis. Statistical analysis was performed using regression analysis and a chi-square test. RESULTS: In human soft-tissue sarcomas, immunohistochemical expression of IL4Rα was significantly associated with IL13Rα1 expression. Nuclear and cytoplasmic expression of IL4Rα and IL13Rα1 were significantly associated with shorter survival of soft-tissue sarcoma patients in univariate analysis. Multivariate analysis indicated that nuclear expression of IL4Rα and IL13Rα1 were independent indicators of shorter overall survival (IL4Rα; p = 0.002, IL13Rα1; p = 0.016) and relapse-free survival (IL4Rα; p = 0.022, IL13Rα1; p < 0.001) of soft-tissue sarcoma patients. Moreover, the co-expression pattern of nuclear IL4Rα and IL13Rα1 was an independent indicator of shorter survival of soft-tissue sarcoma patients (overall survival; overall p < 0.001, relapse-free survival; overall p < 0.001). CONCLUSIONS: This study suggests IL4Rα and IL13Rα1 are associated with the progression of soft-tissue sarcoma, and the expression of IL4Rα and IL13Rα1 might be novel prognostic indicators of soft-tissue sarcoma patients.


Assuntos
Subunidade alfa1 de Receptor de Interleucina-13/metabolismo , Subunidade alfa de Receptor de Interleucina-4/metabolismo , Neoplasias Retroperitoneais/diagnóstico , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Extremidades/patologia , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Prognóstico , Neoplasias Retroperitoneais/metabolismo , Neoplasias Retroperitoneais/patologia , Espaço Retroperitoneal/patologia , Sarcoma/metabolismo , Sarcoma/patologia , Neoplasias de Tecidos Moles/metabolismo , Neoplasias de Tecidos Moles/patologia , Análise de Sobrevida , Adulto Jovem
3.
Urol Int ; 105(1-2): 21-26, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33049748

RESUMO

BACKGROUND: MicroRNA-371a-3p (miR-371), the novel serum biomarker of testicular germ cell tumours (GCTs), is produced by undifferentiated subtypes of GCTs but not by teratoma. Cystic teratoma developing from retroperitoneal metastases of GCT subsequent to chemotherapy had been shown to contain high levels of classical serum tumour markers of GCT in the presence of normal marker levels in serum. To date, no information is available regarding the presence of miR-371 in the cystic fluid of residual teratoma after chemotherapy. METHODS: Four patients (age 18-26 years) undergoing retroperitoneal lymph node dissection (RPLND) for cystic residual masses resulting from chemotherapy of bulky retroperitoneal GCT had measurements of miR-371 in both serum and cystic fluid aspirated from surgical specimens. Measurement of the miR was performed with quantitative real-time PCR using miR-30b-5p as reference. Results were tabulated and analysed in a descriptive manner. RESULTS: Histologically, all of the surgical specimens involved teratoma only with no evidence of vital undifferentiated GCT tissue. All patients were cured. Prior to RPLND, miR-371 serum levels were not measurable or close to zero in all of the patients. Cystic fluid revealed elevated levels of miR-371 in 3 patients and traces of miR in one. CONCLUSIONS: The detection of miR-371 in the cystic fluid of teratoma is somewhat enigmatic since this GCT subtype usually does not express the miR. Two hypotheses may explain the finding: First, miR-371 molecules were released into the cystic fluid by active GCT tissue prior to chemotherapy. High levels were kept after regression of vital GCT tissue because the cystic lumen is without a specific drainage system. Second, teratoma cells lining the interior cyst wall may shed small amounts of miR-371 into the lumen. Because of the lacking drainage system, even small levels may accumulate. The present finding adds to the understanding of the biology of the novel biomarker of GCT.


Assuntos
Biomarcadores Tumorais/análise , Líquido Cístico/química , MicroRNAs/análise , Neoplasias Embrionárias de Células Germinativas/tratamento farmacológico , Segunda Neoplasia Primária/metabolismo , Neoplasias Retroperitoneais/metabolismo , Teratoma/metabolismo , Neoplasias Testiculares/tratamento farmacológico , Adolescente , Adulto , Biomarcadores Tumorais/biossíntese , Humanos , Masculino , MicroRNAs/biossíntese , Adulto Jovem
4.
J Clin Endocrinol Metab ; 106(2): e675-e679, 2021 01 23.
Artigo em Inglês | MEDLINE | ID: mdl-33245336

RESUMO

CONTEXT: Women with congenital adrenal hyperplasia (CAH) may present with androgen excess that is difficult to control with conventional suppressive doses of glucocorticoids. Clinical management is challenging, and the woman is at great risk of developing steroid-induced complications. PATIENTS AND METHODS: A 32-year-old woman with salt-wasting CAH due to 21-hydroxylase deficiency underwent right-sided adrenalectomy because of a large myelolipoma. Over the years, androgens became increasingly difficult to suppress on prednisolone 5 + 0 + 2.5 mg daily, and at age 39 years the left adrenal with an enlarging myelolipoma was removed. A month later serum testosterone levels had increased from 4.1 preoperatively to 18.3 nmol/L (reference 0.2-1.8 nmol/L), and adrenocorticotropin levels from 32 to 283 pmol/L (reference < 14 pmol/L). No adrenal parenchyma was visualized on computed tomography (CT). In the further search for the source of the markedly elevated testosterone, positron emission tomography (PET) was performed with 2 different tracers, 18fluorodeoxyglucose (18FDG) reflecting glucose metabolism and 11C-metomidate, an inhibitor of 11-ß-hydroxylase targeting adrenocortical tissue. RESULTS: 18FDG-PET/CT with cosyntropin stimulation showed ovarian/paraovarian hypermetabolism, suggestive of adrenal rest tumors. Further characterization with 11C-metomidate PET/CT showed uptakes localized to the ovaries/adnexa, behind the spleen, and between the right crus diaphragmaticus and inferior vena cava. CONCLUSION: Adrenal rest tumors can give rise to high androgen levels in spite of suppressive supraphysiological glucocorticoid doses. This case illustrates, for the first time, the value of 11C-metomidate PET as a sensitive method in documenting adrenal rest tumors, currently considered rare in women with CAH.


Assuntos
Hiperplasia Suprarrenal Congênita , Tumor de Resto Suprarrenal/diagnóstico , Neoplasias Ovarianas/diagnóstico , Neoplasias Retroperitoneais/diagnóstico , Hiperplasia Suprarrenal Congênita/complicações , Hiperplasia Suprarrenal Congênita/diagnóstico , Hiperplasia Suprarrenal Congênita/metabolismo , Tumor de Resto Suprarrenal/complicações , Tumor de Resto Suprarrenal/metabolismo , Adulto , Radioisótopos de Carbono , Etomidato/análogos & derivados , Feminino , Humanos , Neoplasias Primárias Múltiplas/complicações , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Primárias Múltiplas/metabolismo , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/metabolismo , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Neoplasias Retroperitoneais/complicações , Neoplasias Retroperitoneais/metabolismo , Sais/metabolismo , Suécia , Desequilíbrio Hidroeletrolítico/complicações , Desequilíbrio Hidroeletrolítico/metabolismo
5.
Br J Haematol ; 190(6): e329-e332, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32572949
6.
Cancer Treat Rev ; 86: 102013, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32278233

RESUMO

Retroperitoneal liposarcomas are rare tumours that carry a poorer prognosis than their extremity counterparts. Within their subtypes - well differentiated (WDL), dedifferentiated (DDL), myxoid (MLS) and pleomorphic (PLS) - they exhibit a diverse genomic landscape. With recent advances in next generation sequencing, the number of studies exploring this have greatly increased. The recent literature has deepened our understanding of the hallmark MDM2/CDK4 amplification in WDL/DDL and addressed concerns about toxicity and resistance when targeting this. The FUS-DDIT3 fusion gene remains the primary focus of interest in MLS with additional potential targets described. Whole genome sequencing has driven identification of novel genes and pathways implicated in WDL/DDL outside of the classic 12q13-15 amplicon. Due to their rarity; anatomical location and histologic subtype are infrequently mentioned when reporting the results of these studies. Reports can include non-adipogenic or extremity tumours, making it difficult to draw specific retroperitoneal conclusions. This narrative review aims to provide a summary of retroperitoneal liposarcoma genomics and the implications for therapeutic targeting.


Assuntos
Lipossarcoma/genética , Neoplasias Retroperitoneais/genética , Animais , Antineoplásicos Alquilantes/uso terapêutico , Aberrações Cromossômicas , Genômica/métodos , Humanos , Lipossarcoma/tratamento farmacológico , Lipossarcoma/metabolismo , Proteínas de Fusão Oncogênica/genética , Fosfatidilinositol 3-Quinases/genética , Fosfatidilinositol 3-Quinases/metabolismo , Neoplasias Retroperitoneais/tratamento farmacológico , Neoplasias Retroperitoneais/metabolismo , Trabectedina/uso terapêutico
8.
Diagn Pathol ; 15(1): 23, 2020 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-32164724

RESUMO

BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS) is a rare fibroblastic tumor often involving deep tissue of trunk and lower extremities in young to middle-aged patients. Rarely, LGFMS can occur in other sites including head and neck, chest, abdomen and female reproductive system. Three cases of LGFMS in mesentery of small intestine have been reported and all have conventional histologic features. Herein we reported a unique case of LGFMS in mesentery of small intestine. CASE PRESENTATION: A 43 year-old male with chief complaint of lower back pain for 4 years presented to our hospital. Physical exam reveal a firm, non-tender, non-distended, mobile large abdominal mass, which was shown on abdominal CT as a 10 cm retroperitoneal tumor. Biopsy revealed a spindle cell neoplasm in a myxoid background with a delicate vascular network. Tumor resection was performed. Gross examination of the resected specimen showed a 10.8 cm, tan-white, smooth, firm, lobulated mesenteric mass with bulging and gelatinous cut surface and confined within small bowel serosa. Microscopic examination demonstrated foci epithelioid cords and whorls with prominent atypia, in additional of regular, bland-appearing spindle cells in a fibrous and myxoid stroma and osseous metaplasia. The tumor cells stained diffusely positive for DOG1 with moderate staining density, and diffusely and strongly positive for MUC4. Rearrangement involving FUS (16p11.2) gene was identified with break-apart probe and confirmed by Anchored Multiplex PCR. A final diagnosis of low-grade fibromyxoid sarcoma was rendered. CONCLUSION: Our case highlights the importance of including LGFMS in the differential diagnosis of mesenteric tumors and the DOG1 positivity which could represent a potential diagnostic pitfall.


Assuntos
Anoctamina-1/metabolismo , Fibroma/patologia , Fibrossarcoma/patologia , Proteínas de Neoplasias/metabolismo , Neoplasias Retroperitoneais/patologia , Adulto , Biomarcadores Tumorais/análise , Fibroma/metabolismo , Fibrossarcoma/metabolismo , Rearranjo Gênico , Humanos , Intestino Delgado/patologia , Masculino , Mesentério/patologia , Proteína FUS de Ligação a RNA/genética , Neoplasias Retroperitoneais/metabolismo
9.
Appl Immunohistochem Mol Morphol ; 28(4): e33-e35, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-28877071

RESUMO

The World Health Organization has recognized Xp11.2 translocation-associated renal cell carcinoma (RCC) as a distinct neoplasm that arises within the kidney. Although many reports of extrarenal carcinoma may be found in the literature, to the best of our knowledge, Xp11 translocation-associated RCC with intact kidneys has not been documented. This report describes a multilobulated right retroperitoneal soft tissue mass (7.9×5.3×12.6 cm) of a 37-year-old man complaining of abdominal pain in the right side. The patient underwent a computed tomography-guided biopsy. Microscopic evaluation reveals a tumor with papillary and sheaths architectures with cells revealing clear to eosinophilic cytoplasm. Immunohistochemical evaluation on the biopsy reveals that the tumor is positive for PAX-8, CD10, and TFE3. It is negative for CK7, EMA, Vimentin, RCC, CK8/18, D20, CD3, PLAP, OCT4, CD30, MART-1, Inhibin, S-100, HMB-45, Desmin, SMA, and DOG-1. The diagnosis was malignant epithelioid neoplasm and the diagnosis of translocation RCC was suggested. Excision was recommended. The patient underwent right radical nephrectomy with removal of this large mass. Pathologic examination showed a large cystic and solid, nonhomogenous mass with some necrotic areas, originating from the perirenal fat between the adrenal gland and the kidney. Microscopic features showed a tumor with papillary, rhabdoid, and clear cell features. Immunohistochemical stains showed that the tumor cells positively expressed AMACR, PAX-8, CD10, RCC, and TFE3, but were negative for cytokeratins, vimentin, HMB-45, desmin, SMA, EMA, and MSA. Cytogenetic studies confirmed the diagnosis of Xp11.2 translocation-associated RCC with positive TFE3 gene rearrangement. To the best of our knowledge, this type of extrarenal tumor has never been reported.


Assuntos
Carcinoma de Células Renais , Cromossomos Humanos X/genética , Neoplasias Renais , Proteínas de Neoplasias , Neoplasias Retroperitoneais , Translocação Genética , Adulto , Carcinoma de Células Renais/genética , Carcinoma de Células Renais/metabolismo , Carcinoma de Células Renais/patologia , Humanos , Hibridização in Situ Fluorescente , Neoplasias Renais/genética , Neoplasias Renais/metabolismo , Neoplasias Renais/patologia , Masculino , Proteínas de Neoplasias/genética , Proteínas de Neoplasias/metabolismo , Neoplasias Retroperitoneais/genética , Neoplasias Retroperitoneais/metabolismo , Neoplasias Retroperitoneais/patologia
10.
Clin Nucl Med ; 44(12): 991-992, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31689283

RESUMO

A 48-year-old woman with intermittent lower back pain for 9 months and known retroperitoneal neurofibroma underwent F-NaF PET/CT scan to assess possible bony lesions causing the pain. Incidentally, the images showed elevated NaF activity in the retroperitoneal neurofibroma. In addition, uterine leiomyoma with heterogeneous calcifications revealed increased NaF activity.


Assuntos
Calcinose/complicações , Radioisótopos de Flúor , Leiomioma/complicações , Neurofibroma/metabolismo , Neoplasias Retroperitoneais/metabolismo , Fluoreto de Sódio/metabolismo , Transporte Biológico , Humanos , Masculino , Pessoa de Meia-Idade , Neurofibroma/complicações , Neurofibroma/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias Retroperitoneais/complicações , Neoplasias Retroperitoneais/diagnóstico por imagem
11.
Cell Death Dis ; 10(6): 430, 2019 06 03.
Artigo em Inglês | MEDLINE | ID: mdl-31160581

RESUMO

Unraveling the noncoding RNA expression networks governing cancer initiation and development is essential while remains largely uncompleted in retroperitoneal liposarcoma (RLS). Through RNA-seq technologies and computational biology, deregulated long noncoding RNAs (lncRNAs) are being identified and reveal that lncRNAs are implicated in serial steps of RLS development. High-throughput sequencing with computational methods for assembling the transcriptome of five paired RLS patient's tissues. We found that long intergenic noncoding RNA 423 (linc00423) was downregulated in RLS tissues. Gain-of-function assays revealed that overexpressed linc00423 obviously inhibited RLS cell growth in vitro and in vivo. Additionally, RNA sequence, RNA-pulldown and RIP assays evidenced that linc00423 involved in MAPK signaling pathway via destabilizing of nuclear factor of activated T-cells 3 (NFATC3). Summing up, our findings demonstrated that linc00423 acted as the tumor suppressor in RLS cells through regulating the protein level of NFATC3 at a post-transcriptional level and negatively regulated the MAPK signaling pathway at a transcriptional level. Linc00423 might serve as a candidate prognostic biomarker and a target for novel therapies of RLS patients.


Assuntos
Genes Supressores de Tumor , Lipossarcoma/metabolismo , Fatores de Transcrição NFATC/metabolismo , RNA Longo não Codificante/metabolismo , Neoplasias Retroperitoneais/metabolismo , Animais , Proliferação de Células/genética , Feminino , Regulação Neoplásica da Expressão Gênica , Células HEK293 , Humanos , Lipossarcoma/genética , Sistema de Sinalização das MAP Quinases/genética , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Nus , Fatores de Transcrição NFATC/genética , Ligação Proteica , RNA Longo não Codificante/genética , RNA-Seq , Neoplasias Retroperitoneais/genética , Transplante Heterólogo , Regulação para Cima/genética
12.
Pediatr Blood Cancer ; 66(9): e27825, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31135092

RESUMO

Neonatal neuroblastoma may require chemotherapy either due to mass effect or unfavourable cytogenetics. This case focuses on using pharmacokinetic (PK) guided chemotherapy to treat neonatal neuroblastoma. A newborn baby was noted to have left leg immobility. Imaging showed a retroperitoneal tumour with spinal canal extension causing spinal cord compression. PK-guided carboplatin was given after conventionally dosed chemotherapy demonstrated no improvement. After initiation of PK therapy, clinical and radiological improvement was seen. We discuss our decision to use PK-guided chemotherapy despite guidelines recommending weight-based dosing and discuss the benefits in terms of clinical efficacy without increased toxicity.


Assuntos
Carboplatina , Recém-Nascido Prematuro , Neuroblastoma , Neoplasias Retroperitoneais , Compressão da Medula Espinal , Carboplatina/administração & dosagem , Carboplatina/farmacocinética , Feminino , Humanos , Recém-Nascido , Neuroblastoma/diagnóstico por imagem , Neuroblastoma/tratamento farmacológico , Neuroblastoma/metabolismo , Neoplasias Retroperitoneais/diagnóstico por imagem , Neoplasias Retroperitoneais/tratamento farmacológico , Neoplasias Retroperitoneais/metabolismo , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/tratamento farmacológico , Compressão da Medula Espinal/metabolismo
13.
Hum Pathol ; 86: 66-75, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30529752

RESUMO

Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are neuroendocrine tumors that express somatostatin receptors (SSTRs), a phenomenon that constitutes a basis for tumor imaging and treatment with somatostatin analogues and peptide receptor radionuclide therapy. We studied the immunohistochemical expression of SSTR1-5 in 151 primary tumors, including 14 metastasized and 16 SDHB-deficient tumors. SSTR2 and SSTR3 were most abundantly present in these tumors, whereas the tumors were mostly negative for SSTR1, SSTR4, and SSTR5. All metastasized PGLs (9/9), but only one metastasized PHEO (1/5), were strongly SSTR2 positive. SSTR3 expression was lower in metastatic tumors and tumors with a high proliferation rate (MIB1 ≥ 5%), but tumors had variable individual SSTR profiles. No correlation was found between SDHB status and SSTR expression. Our results suggest that new SSTR analogues with affinity for several SSTRs could be relevant for a subgroup of patients with these tumors. Better knowledge of tumor SSTR profiles could open the door for personalized imaging and treatment in the future. Because SSTR profiles vary in PHEOs and PGLs, individual analysis is required for each tumor.


Assuntos
Neoplasias das Glândulas Suprarrenais/metabolismo , Paraganglioma/metabolismo , Feocromocitoma/metabolismo , Receptores de Somatostatina/metabolismo , Neoplasias Retroperitoneais/metabolismo , Neoplasias das Glândulas Suprarrenais/patologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Paraganglioma/patologia , Feocromocitoma/patologia , Neoplasias Retroperitoneais/patologia , Adulto Jovem
14.
Zhonghua Zhong Liu Za Zhi ; 40(4): 258-263, 2018 Apr 23.
Artigo em Chinês | MEDLINE | ID: mdl-29730911

RESUMO

Objective: To investigate the expression and prognostic value of alpha smooth muscle actin(α-SMA) and Ki-67 in retroperitoneal leiomyosarcoma. Methods: Fifty retroperitoneal leiomyosarcoma patients who underwent operation in Chinese People's Liberation Army General Hospital from May 2002 to December 2015 were retrospectively analyzed. There were 14 males and 36 females form 21 to 79 and an average age of 48. Kaplan-Meier estimations and Cox regression analyses were performed. Results: Of the 50 cases, 45 patients underwent complete resection, and others are not. The overall 1, 3, 5-year survival rates were 86.0%, 46.0% and 28.0%, respectively. Tumor size, extent of resection, pathological stage, and expression levels of Ki-67 and alpha smooth muscle actin (α-SMA) were closely related to the survival of retroperitoneal leiomyosarcoma patients (all P<0.05), respectively. Multivariate analysis showed that pathological grade and degree of surgical resection were independent risk factors in the prognosis of patients (P<0.05). Conclusion: The high expression of α-SMA and Ki-67 are indicators of poor prognosis in retroperitoneal leiomyosarcoma, which can be used as a potential survival predictor in patients with retroperitoneal leiomyosarcoma.


Assuntos
Actinas/metabolismo , Antígeno Ki-67/metabolismo , Leiomiossarcoma/metabolismo , Leiomiossarcoma/mortalidade , Proteínas de Neoplasias/metabolismo , Neoplasias Retroperitoneais/metabolismo , Neoplasias Retroperitoneais/mortalidade , Adulto , Idoso , Feminino , Humanos , Estimativa de Kaplan-Meier , Leiomiossarcoma/patologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Análise de Regressão , Neoplasias Retroperitoneais/patologia , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Fatores de Tempo , Adulto Jovem
16.
Anticancer Res ; 38(4): 1903-1909, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29599305

RESUMO

BACKGROUND/AIM: The objective of the present study was to determine the clinicopathological factors and treatment outcomes of patients suffering from mesenteric or retroperitoneal extragastrointestinal stromal tumors (EGISTs). MATERIALS AND METHODS: A detailed search in PubMed, using the key words "extragastrointestinal stromal tumors" and "EGIST", found eight studies fulfilling the criteria of this study. RESULTS: Thirty-six patients with a mesenteric and 24 patients with a retroperitoneal EGIST were analyzed, with a follow-up period ranging from 2 to 192 months. Retroperitoneal tumors presented as larger tumors than mesenteric ones, with 95% and 93% immunohistochemical positivity for CD117 antigen, respectively. Surgical resection was performed in 91% of cases, with 57% of patients with mesenteric and 70% of patients with retroperitoneal EGISTs being alive at the last follow-up. CONCLUSION: EGISTs most commonly are of considerable size and usually with a high mitotic count, rendering them high-risk tumors. Tumor necrosis, nuclear atypia, tumor histology, and mutations in the tyrosine kinase KIT or platelet-derived growth factor receptor A (PDGFRA) gene, seem to influence tumor behavior.


Assuntos
Mesentério/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias de Tecido Conjuntivo/patologia , Neoplasias Retroperitoneais/patologia , Células Estromais/patologia , Humanos , Imuno-Histoquímica , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/metabolismo , Neoplasias de Tecido Conjuntivo/genética , Neoplasias de Tecido Conjuntivo/metabolismo , Neoplasias de Tecido Conjuntivo/cirurgia , Neoplasias Retroperitoneais/genética , Neoplasias Retroperitoneais/metabolismo , Neoplasias Retroperitoneais/cirurgia , Fatores de Risco , Resultado do Tratamento
17.
Am J Clin Pathol ; 149(2): 128-134, 2018 Jan 29.
Artigo em Inglês | MEDLINE | ID: mdl-29385413

RESUMO

Objectives: Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor often arising in the lower extremities. Only rare examples in the abdominal cavity, pelvis, and retroperitoneum have been reported. Methods: cases of abdominopelvic and retroperitoneal LGFMS were retrieved. MUC4, Actin, ALK, ß-catenin, desmin, DOG1, KIT, S100 protein, and STAT6 testing was performed, and a subset was tested for FUS rearrangement. Results: Sites included intra-abdominal/abdominal wall (four cases), retroperitoneum (three cases), pelvis (three cases), small bowel (two cases), and kidney (one case) (median size, 15 cm; age range, 5-61 years). Tumors harbored spindled cells with mild to moderate atypia, displaying alternating myxoid nodules and hyalinized areas. All cases were positive for MUC4, and five (of five) cases tested harbored FUS rearrangement. Variable positivity for DOG1 (four of 10) and actin (two of 10) was identified. Six tumors recurred, and one patient developed metastasis. Conclusion: LGFMS arising in these central locations exhibits similar clinicopathologic features to its counterpart in the extremities. LGFMS at these sites may show limited immunoreactivity for DOG1 and actin.


Assuntos
Biomarcadores Tumorais/metabolismo , Fibrossarcoma/patologia , Neoplasias Pélvicas/patologia , Neoplasias Retroperitoneais/patologia , Adolescente , Adulto , Feminino , Fibrossarcoma/diagnóstico , Fibrossarcoma/metabolismo , Seguimentos , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Neoplasias Pélvicas/diagnóstico , Neoplasias Pélvicas/metabolismo , Prognóstico , Neoplasias Retroperitoneais/diagnóstico , Neoplasias Retroperitoneais/metabolismo
19.
Cancer Cytopathol ; 125(8): 604-614, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28805986

RESUMO

BACKGROUND: Histiocytic sarcoma (HS) is a rare malignant neoplasm showing morphologic and immunophenotypic features of histiocytes. Molecular characteristics of HS and fine-needle aspiration (FNA) criteria for its diagnosis have not been established. METHODS: A case series of HS in 8 FNA samples from 6 patients was reviewed along with histopathologic and clinical data. Immunohistochemistry was performed on cell blocks (3 cases), core biopsies (5 cases), and surgical specimens (4 cases). Targeted-exome next-generation sequencing (NGS) was performed on surgical resection specimens in 4 cases. RESULTS: Four patients had a known history of hematolymphoid malignancy. Cytomorphologic features included variably cellular smears composed of large epithelioid cells with reniform nuclei and abundant vacuolated cytoplasm, in an inflammatory background, with occasional cytophagocytosis and lymphoglandular bodies. Marked pleomorphism, multinucleated monster cells, and binucleated histiocytoid cells with partially overlapping, eccentrically placed nuclei resembling Pac-Man were common. Most cases expressed histiocytic markers CD68 (6 of 7 cases), CD163 (5 of 5 cases), and PU.1 (3 of 4 cases). In 3 cases, NGS analysis revealed alterations in lysine methyltransferase 2D (KMT2D)/mixed-lineage leukemia 2 (MLL2), a gene involved in chromatin regulation and previously implicated in the pathogenesis of follicular lymphoma. CONCLUSIONS: Although diagnosing HS with FNA alone is extremely challenging, the presence of pleomorphic and epithelioid large cells with binucleation and/or multinucleation in an inflammatory background should prompt the diagnosis of HS with judicious use of confirmatory histiocytic lineage markers. The detection of recurrent KMT2D/MLL2 alterations implicates epigenetic regulation in the pathogenesis of HS and supports the notion of transdifferentiation from a genetically similar but phenotypically distinct tumor of a different lineage. Cancer Cytopathol 2017;125:604-14. © 2017 American Cancer Society.


Assuntos
Neoplasias Ósseas/patologia , Sarcoma Histiocítico/patologia , Neoplasias Hepáticas/patologia , Neoplasias Pulmonares/patologia , Neoplasias Retroperitoneais/patologia , Escápula/patologia , Neoplasias de Tecidos Moles/patologia , Neoplasias Uterinas/patologia , Idoso , Idoso de 80 Anos ou mais , Antígenos CD/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Neoplasias Ósseas/genética , Neoplasias Ósseas/metabolismo , Neoplasias Ósseas/cirurgia , Proteínas de Ligação a DNA/genética , Epigênese Genética , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Sarcoma Histiocítico/genética , Sarcoma Histiocítico/metabolismo , Sarcoma Histiocítico/cirurgia , Humanos , Imuno-Histoquímica , Neoplasias Hepáticas/metabolismo , Neoplasias Hepáticas/secundário , Neoplasias Hepáticas/cirurgia , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/metabolismo , Neoplasias Pulmonares/cirurgia , Masculino , Pessoa de Meia-Idade , Pescoço , Proteínas de Neoplasias/genética , Proteínas Proto-Oncogênicas/metabolismo , Receptores de Superfície Celular/metabolismo , Neoplasias Retroperitoneais/genética , Neoplasias Retroperitoneais/metabolismo , Neoplasias Retroperitoneais/cirurgia , Escápula/metabolismo , Escápula/cirurgia , Análise de Sequência de DNA , Neoplasias de Tecidos Moles/metabolismo , Neoplasias de Tecidos Moles/secundário , Neoplasias de Tecidos Moles/cirurgia , Transativadores/metabolismo , Neoplasias Uterinas/genética , Neoplasias Uterinas/metabolismo , Neoplasias Uterinas/cirurgia
20.
PLoS One ; 12(8): e0182660, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28783764

RESUMO

BACKGROUND: Patients with inoperable esophageal squamous cell carcinoma (ESCC) were not homogeneous and their outcomes were widely divergent. There was a lack of identified clinical factors related to prognosis; and there were no previous studies constructing prognosis score to predict survival and guide treatment. METHODS: In this retrospective cohort study, twelve clinical characteristics of one hundred and twenty inoperable ESCC patients were collected at diagnosis and analyzed by Cox regression model. Various methods including univariate analysis, confounding adjusted multivariate analysis and model selection were applied to determine factors associated with poor prognosis; and prognosis score was built on established factors. RESULTS: Four characters were identified as poor prognosis factors, including mid- and low-thoracic tumor (aHR = 2.20, 95% CI = 1.03, 4.72), abdominal and retroperitoneal lymph node metastasis (aHR = 1.62, 95% CI = 1.00, 2.64), albumin no more than 39g/L (aHR = 2.81, 95% CI = 1.24, 6.41) and hematogenous metastasis (aHR = 1.61, 95% CI = 0.97, 2.69). Patients were stratified into three groups by prognosis score, that was, good survival with none of four identified factors (score zero), poor survival with three to four factors (score three to four) and median with one to two factors (score one to two), survival of three groups were statistically different (ptrend = 0.020). CONCLUSION: Prognosis score based on selected clinical characteristics could predict survival among inoperable ESCC patients, which was critical for individualized treatment and central of precise medicine.


Assuntos
Carcinoma de Células Escamosas/complicações , Carcinoma de Células Escamosas/patologia , Neoplasias Esofágicas/complicações , Neoplasias Esofágicas/patologia , Adulto , Idoso , Biomarcadores Tumorais/metabolismo , Carcinoma de Células Escamosas/metabolismo , Carcinoma de Células Escamosas/cirurgia , Neoplasias Esofágicas/metabolismo , Neoplasias Esofágicas/cirurgia , Carcinoma de Células Escamosas do Esôfago , Esofagectomia , Feminino , Hemoglobinas/metabolismo , Humanos , Estimativa de Kaplan-Meier , Metástase Linfática/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Neoplasias Retroperitoneais/metabolismo , Neoplasias Retroperitoneais/patologia , Neoplasias Retroperitoneais/secundário , Neoplasias Retroperitoneais/cirurgia , Estudos Retrospectivos
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