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1.
J Med Case Rep ; 15(1): 484, 2021 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-34593019

RESUMO

BACKGROUND: Myxofibrosarcoma (MFS) is a rare soft tissue sarcoma with a high recurrence rate and a low risk of distant metastasis. It occurs mainly in the extremities of elderly men. Head and neck MFS is extremely rare. Surgery is the cornerstone of treatment. The role of radiotherapy (RT) and chemotherapy (CHT) on MFS is still debated. CASE PRESENTATION: A 67-year-old Caucasian man presented to our sarcoma referral center (SRC) with a history of MFS of the neck excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis. After a multidisciplinary discussion, preoperative RT was administered with a total dose of 50 Gy followed by wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred during the 24-month postoperative follow-up. CONCLUSIONS: The case described suggests the importance of planned combined treatments with both RT and surgery for high-grade soft tissue sarcoma. RT seems to be promising within this specific histotype. Close follow-up is advisable in all cases. Further studies are needed to confirm if the observed efficacy of combined treatments results in a prolonged time of disease-free survival and overall survival.


Assuntos
Fibrossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Idoso , Fibrossarcoma/diagnóstico por imagem , Fibrossarcoma/cirurgia , Humanos , Masculino , Margens de Excisão , Recidiva Local de Neoplasia , Estudos Retrospectivos , Neoplasias de Tecidos Moles/cirurgia
2.
J Med Case Rep ; 15(1): 501, 2021 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-34625114

RESUMO

BACKGROUND: Myeloid sarcoma is a solid tumor that consists of immature myeloid cells occurring at an extramedullary site. It can present before, concurrent with, or after the diagnosis of acute myeloid leukemia or other myeloproliferative diseases, and a proportion of patients never develop bone marrow infiltration. Only a few isolated cases of pediatric orbital myeloid sarcoma have been reported, and they are often associated with a high misdiagnosis rate. CASE REPORT: We report a rare case of pediatric orbital myeloid sarcoma associated with blunt trauma in a 3-year-old Caucasian male patient, which was clinically and radiologically misdiagnosed for orbital subperiostal hematoma. The patient underwent a surgical intervention to drain the hematoma when an orbital mass was found. The microscopic, immunologic, and genetic features of the tumor and the myelogram were in favor of LAM2, and the patient was started with chemotherapy with a favorable evolution within 18 months follow-up. CONCLUSION: Orbital myeloid sarcoma usually exhibits clinical and radiological features that can be easily misleading, especially if it happens de novo or as the first manifestation of acute myeloid leukemia. Only a few isolated cases have reported and proposed trauma as a trigger event of the onset of this type of tumor proliferation, but further investigations and evidence are needed to support this hypothesis.


Assuntos
Neoplasias Orbitárias , Sarcoma Mieloide , Neoplasias de Tecidos Moles , Criança , Pré-Escolar , Erros de Diagnóstico , Hematoma , Humanos , Masculino , Neoplasias Orbitárias/diagnóstico , Sarcoma Mieloide/diagnóstico por imagem
3.
J Pak Med Assoc ; 71(Suppl 5)(8): S75-S78, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34634021

RESUMO

OBJECTIVE: To study the frequency of the thigh, hip and groin soft tissue sarcomas and retrospectively analyse the management, treatment results, and outcomes of these uncommon malignant tumours, in a tertiary care hospital of the city of Karachi. Methodology: Data of soft tissue tumours registered from 2017-2018 was retrieved during January 2019 to March 2019 from Aga Khan University Hospital, Karachi bone and soft tissue tumour registry. A retrospective review was performed and all soft tissue tumour cases treated with surgical intervention (with adjuvant /neoadjuvant therapy) or palliative intention were included. RESULTS: Total 119 cases of soft tissue tumours (STS) were identified out of which 85 were malignant cases (sarcomas) while 30 were benign. On presentation 84 (70.6%) were primary cases. On topographical distribution, there were 25 patients who had hip, groin and thigh sarcoma. Of these, 15 were males and 10 were females. As treatment, neo-adjuvant radiation was done in 4 (16%) patients and adjuvant chemo/radio therapy was given to 13 (52%) patients. Wide margin excision was performed in 19 (76%) patients and 4 (16%) had amputation. Reconstruction was offered to 3 (12%) patients. In post-surgical complications, 1 (4%) patient had wound infection. On final surgical histopathology, majority of the sarcomas were liposarcomas, myxofibrosarcoma, synovial sarcoma and Leiomyosarcoma. Post-surgery recurrence occurred in 7 (28%) patients. Overall survival was 76%. CONCLUSIONS: In treatment of soft tissue sarcoma, limb salvage is an achievable option and survival results are also good.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Feminino , Virilha , Humanos , Masculino , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/terapia , Estudos Retrospectivos , Sarcoma/epidemiologia , Sarcoma/terapia , Neoplasias de Tecidos Moles/epidemiologia , Neoplasias de Tecidos Moles/terapia , Centros de Atenção Terciária , Coxa da Perna , Resultado do Tratamento
4.
J Med Case Rep ; 15(1): 525, 2021 Oct 18.
Artigo em Inglês | MEDLINE | ID: mdl-34663426

RESUMO

BACKGROUND: Desmoplastic fibroblastoma (also known as collagenous fibroma) is a benign, slowly growing soft-tissue tumor. Most desmoplastic fibroblastomas develop in the limbs, neck, or trunk. A mediastinal origin is quite rare. CASE PRESENTATION: A 32-year-old Asian female was referred to us for the diagnosis and treatment of an anterior mediastinal tumor. The tumor was 80 mm in the largest diameter and was located on the pericardium. No invasion was evident. She underwent resection of the tumor via video-assisted thoracoscopic resection. The tumor was totally encapsulated, and its pedicle was on the pericardium. The resected specimen was very rigid, making it difficult to remove from the intercostal space. Histologically, the tumor was composed of a paucicellular dense collagenous tissue. Mitosis was rarely observed, and cellular atypia was not evident, suggesting that the tumor was benign. We diagnosed the tumor as a desmoplastic fibroblastoma by morphology and immunohistochemistry. CONCLUSIONS: Desmoplastic fibroblastoma of the mediastinum is an extremely rare disease. Preoperative diagnosis is difficult. Early surgical resection is suitable for diagnosis and treatment planning.


Assuntos
Fibroma Desmoplásico , Neoplasias de Tecidos Moles , Parede Torácica , Adulto , Feminino , Humanos , Imuno-Histoquímica , Mediastino/diagnóstico por imagem , Mediastino/cirurgia
5.
Am J Case Rep ; 22: e932514, 2021 Oct 20.
Artigo em Inglês | MEDLINE | ID: mdl-34669689

RESUMO

BACKGROUND Alveolar soft-part sarcoma is an uncommon mesenchymal tumor accounting for approximately 0.7% of soft tissue sarcomas in adults. It mainly affects young adults, with a peak incidence between 15 and 35 years old. Available data indicate that surgical resection with adjuvant therapy using tyrosine kinase inhibitor may be considered the standard treatment. The rarity of the disease and resultant data scarcity makes it difficult to establish treatment guidelines. CASE REPORT We present the 9-year follow-up of a 24-year-old patient with an initially advanced (stage IV), huge, 21-cm alveolar soft-part sarcoma of the retroperitoneum. During the observation period, the patient developed pulmonary, brain, and bone metastases. In the course of treatment, she underwent excision of the main tumor, excision of satellite tumors, and brain metastasectomies, and was treated with sunitinib, pazopanib, and radiotherapy. No similar case reports were found in the PubMed database. CONCLUSIONS Our multimodal approach resulted in a long period of stable disease. Late progression may occur; therefore, frequent and thorough imaging evaluation of such patients is crucial. Our case is one of the largest ASPS tumors reported, and her long-term successful treatment makes this report valuable, considering the scarcity of data regarding treatment of ASPS. Further large-cohort, multi-center studies are necessary to establish the best treatment.


Assuntos
Neoplasias Ósseas , Sarcoma Alveolar de Partes Moles , Neoplasias de Tecidos Moles , Adolescente , Adulto , Feminino , Seguimentos , Humanos , Sarcoma Alveolar de Partes Moles/cirurgia , Neoplasias de Tecidos Moles/terapia , Adulto Jovem
6.
Arkh Patol ; 83(5): 31-38, 2021.
Artigo em Russo | MEDLINE | ID: mdl-34609802

RESUMO

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of intermediate malignancy potential, which predominantly affects children and young adults. This paper describes two cases of AFH, as well as a review of literature during 1979 to 2021. It gives data on the epidemiology, clinical features, diagnosis, and genetic characteristics of AFH.


Assuntos
Histiocitoma Fibroso Benigno , Histiocitoma Fibroso Maligno , Neoplasias de Tecidos Moles , Criança , Histiocitoma Fibroso Benigno/diagnóstico , Histiocitoma Fibroso Benigno/genética , Histiocitoma Fibroso Maligno/diagnóstico , Histiocitoma Fibroso Maligno/genética , Humanos , Adulto Jovem
9.
World J Surg Oncol ; 19(1): 267, 2021 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-34479594

RESUMO

BACKGROUND: Extraskeletal osteosarcoma (ESOS) is a rare mesenchymal malignancy, which produces osteoid, bone, or chondroid material and is located in the soft tissue without attachment to skeletal bones and periosteum. One of the things that ESOS originated from mesentery is much rarer. CASE PRESENTATION: A 75-year female had a history of pain in the left lower abdomen for more than 4 months. Abdominal computerized tomography (CT) and magnetic resonance imaging revealed a large, irregular, and solid-cystic mass (largest diameter was 11.5 cm). The tumor was radically removed during an open operation. It was composed of abundant osteoid and polyhedral-shaped tumor cells with high atypia and high mitotic activity microscopically. The final pathological diagnosis was osteoblastic osteosarcoma, arising from the sigmoid mesocolon with negative margins. A 9-month follow-up by CT exhibited signs of peritoneal metastasis. CONCLUSIONS: Given the rarity of cases of mesenteric ESOS, diagnosis mainly depended on pathology findings or should be taken into consideration when the mesenteric mass was found. Its most effective treatment had not been determined, with surgical excision being generally accepted. Ensuring negative surgical margins may be an important factor affecting prognosis.


Assuntos
Neoplasias Ósseas , Mesocolo , Osteossarcoma , Neoplasias de Tecidos Moles , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/cirurgia , Feminino , Humanos , Mesocolo/diagnóstico por imagem , Mesocolo/cirurgia , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/cirurgia , Prognóstico
10.
Bone Joint J ; 103-B(9): 1541-1549, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34465152

RESUMO

AIMS: While a centralized system for the care of patients with a sarcoma has been advocated for decades, regional variations in survival remain unclear. The aim of this study was to investigate regional variations in survival and the impact of national policies in patients with a soft-tissue sarcoma (STS) in the UK. METHODS: The study included 1,775 patients with a STS who were referred to a tertiary sarcoma centre. The geographical variations in survival were evaluated according to the periods before and after the issue of guidance by the National Institute for Health and Care Excellence (NICE) in 2006 and the relevant evolution of regional management. RESULTS: There had been a significant difference in survival between patients referred from the North East, North West, East Midlands, West Midlands, South West, and Wales in the pre-NICE era (five-year disease-specific survival (DSS); South West, 74% vs North East, 47% (p = 0.045) or West Midlands, 54% (p = 0.049)), which was most evident for patients with a high-grade STS. However, this variation disappeared in the post-NICE era, in which the overall DSS for high-grade STS improved from 47% to 68% at five years (p < 0.001). Variation in the size of the tumour closely correlated with the variation in DSS, and the overall size of the tumour and incidence of metastasis at the time of diagnosis also decreased after the national policies were issued. CONCLUSION: The survival of patients with a STS improved and regional variation corrected after the introduction of national policies, as a result of a decreasing size of tumour and incidence of metastasis at the time of diagnosis, particularly in patients with a high-grade STS. This highlights the positive impact of national guidelines on regional variation in the presentation, management, and outcome in patients with a STS. Cite this article: Bone Joint J 2021;103-B(9):1541-1549.


Assuntos
Extremidades , Política de Saúde , Sarcoma/mortalidade , Neoplasias de Tecidos Moles/mortalidade , Neoplasias Torácicas/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Metástase Neoplásica , Características de Residência , Estudos Retrospectivos , Medicina Estatal , Taxa de Sobrevida , Reino Unido/epidemiologia
12.
Ugeskr Laeger ; 183(35)2021 08 30.
Artigo em Dinamarquês | MEDLINE | ID: mdl-34477087

RESUMO

Primary cutaneous sarcomas are a heterogenous group of malignant soft tissue tumours arising in the dermal and subcutaneous layers of the skin. These tumours are rare and have a variety of clinical manifestations. They differ from deep soft tissue sarcomas in terms of prognosis, which for most types of cutaneous sarcomas is favourable. The tumours are primarily managed by complete surgical excision with margin control. The purpose of this review is to present some of these rare tumors and describe their management.


Assuntos
Neoplasias da Mama , Neoplasias de Tecido Conjuntivo , Sarcoma , Neoplasias Cutâneas , Neoplasias de Tecidos Moles , Feminino , Humanos , Sarcoma/diagnóstico , Sarcoma/cirurgia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/cirurgia
13.
Tidsskr Nor Laegeforen ; 141(2021-12)2021 09 07.
Artigo em Norueguês | MEDLINE | ID: mdl-34505491

RESUMO

For locally advanced soft tissue sarcomas and metastases from melanoma located in the extremities, mutilating surgery or amputation may be necessary to achieve local control. Isolated limb perfusion with high-dose chemotherapy may represent an alternative to amputation for this patient group.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Quimioterapia do Câncer por Perfusão Regional , Extremidades , Humanos , Perfusão , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico
14.
BMJ Case Rep ; 14(9)2021 Sep 20.
Artigo em Inglês | MEDLINE | ID: mdl-34544702

RESUMO

Giant cell tumour of tendon sheath also known as benign synovioma is a slow-growing benign tumour originating from tendon sheath, ligaments or bursa. We present a case of swelling over the left foot of 7-month duration in 11-year-old boy diagnosed as giant cell tumour of tendon sheath. There was an extensive pressure effect of tumour mass on the second metatarsal evident by scalloping. Local excision was planned and executed, and reduction in scalloping was evident at 26-month follow-up with no recurrence. We conclude that en bloc resection of tumour with a hydrogen peroxide lavage may result in a favourable prognosis without recurrence.


Assuntos
Tumores de Células Gigantes , Neoplasias de Tecidos Moles , , Tumores de Células Gigantes/diagnóstico por imagem , Tumores de Células Gigantes/cirurgia , Humanos , Lactente , Masculino , Recidiva Local de Neoplasia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/cirurgia , Tendões/cirurgia
15.
Eur J Radiol ; 143: 109934, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34500411

RESUMO

PURPOSE: To evaluate and compare the diagnostic performances of whole-lesion apparent diffusion coefficient (ADC) histogram analysis and single-slice ADC measurement in the differentiation of benign and malignant soft tissue tumors. METHODS: Fifty-three patients (mean age: 48.5 ± 21.4) with soft tissue tumors (27 benign and 26 malignant) were evaluated with diffusion-weighted MRI. Whole-lesion ADC histogram parameters (mean, median, 10th percentile, 90th percentile, minimum, maximum, range, mean absolute deviation, interquartile range, kurtosis, skewness, root mean squared, variance and inhomogeneity) of the lesions were measured using the whole solid tumor volume region of interest (ROI). In other sessions, five ROIs were manually drawn on the tumor slices, and mean ADC and minimum ADC of the measurements were calculated. Diagnostic accuracies of the two methods were assessed and compared. RESULTS: Mean, median, minimum, 10th percentile, 90th percentile, range, root mean squared and inhomogeneity of ADC histogram analysis, and mean ADC and minimum ADC of single-slice ADC measurement were significantly different between malignant and benign lesions (p < 0.001 - p = 0.002). Among the ADC histogram parameters, 10th percentile had the highest diagnostic performance (AUC = 0.825) followed by mean (AUC = 0.792) and median (AUC = 0.789). For the single-slice ADC measurement, the AUC of mean ADC and minimum ADC were 0.842 and 0.786, respectively. Mean ADC of single-slice measurement had a similar diagnostic performance with the 10th percentile, mean, and median of ADC histogram analysis (p = 0.070-1.000). CONCLUSIONS: Both whole-lesion ADC histogram analysis and single-slice ADC measurement can differentiate benign and malignant soft tissue tumors with similar diagnostic performances.


Assuntos
Interpretação de Imagem Assistida por Computador , Neoplasias de Tecidos Moles , Adulto , Idoso , Imagem de Difusão por Ressonância Magnética , Humanos , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade , Neoplasias de Tecidos Moles/diagnóstico por imagem
16.
Eur J Radiol ; 143: 109937, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34547634

RESUMO

The WHO classification of soft tissue tumours categorizes the lesions based on histological differentiation. In the recent fifth edition (2020), many new entities have been introduced, based on their distinct biological behaviour, genetics and morphology. With the recent advances and better understanding of molecular genetics, several novel recurrent genetic alterations have been incorporated in this edition. These may serve as reliable diagnostic and prognostic markers for various soft tissue tumours. Though predominantly a pathologist's game; the radiologists must be aware of the recent updates to contribute towards the optimal diagnosis and management of soft tissue tumours. The imaging features of the new entities have not yet been described but are expected to be explored in the coming years. We hereby present a concise review of the updates in the recent WHO classification of soft tissue tumours. We have tried to emphasize the novel molecular genetics and their surrogate immunohistochemical markers which have brought a paradigm shift in the diagnosis, therapeutics, and prognosis of soft tissue tumours. We also present simplified diagnostic algorithms to characterize soft tissue tumours based on the clinico-radiological features.


Assuntos
Neoplasias de Tecidos Moles , Diferenciação Celular , Humanos , Prognóstico , Radiologistas , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/genética , Organização Mundial da Saúde
17.
Ann Diagn Pathol ; 54: 151800, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34464935

RESUMO

BACKGROUND: Challenging emerging entities with distinctive molecular signatures may benefit from algorithms for diagnostic work-up. METHODS: Fusion sarcomas (2020-2021, during pandemic) were diagnosed by clinicoradiology, morphology, phenotype, and next-generation sequencing (NGS). RESULTS: Six fusion sarcomas in two males and four females involved the chest-wall, neck, or extremities; ages ranged 2-73, median 18 years. Sizes ranged 5.3-25.0, median 9.1 cm. These include high grade 1) TPR-NTRK1 of proximal femur with a larger rounded soft tissue mass, previously considered osteosarcoma yet without convincing tumor matrix. A pathologic fracture necessitated emergency hemipelvectomy (NED) and 2) novel KANK1-NTRK2 sarcoma of bone and soft tissue with spindled pleomorphic to epithelioid features (AWD metastases). 3) Novel ERC1-ALK unaligned fusion, a low grade infiltrative deep soft tissue hand sarcoma with prominent-vascularity, myopericytoid/lipofibromatosis-like ovoid cells, and collagenized stroma, was successfully treated with ALK-inhibitor (Crizotinib), avoiding amputation. These NTRK and ALK tumors variably express S100 and CD34 and were negative for SOX10. 4) and 5) CIC-DUX4 round cell tumors (rapid metastases/demise), one with COVID superinfection, were previously treated as Ewing sarcoma. These demonstrated mild pleomorphism and necrosis, variable myxoid change and CD99 reactivity, and a distinctive dot-like-Golgi WT1 immunostaining pattern. 6) A chest wall/thoracic round cell sarcoma, focal CD34/ keratins/CK7, revealed nuclear-STAT6, STAT6-NAB2 by NGS, confirming malignant solitary fibrous tumor, intermediate-risk-stratification (AWD metastases). CONCLUSIONS: Recent fusion sarcomas include new KANK1-NTRK2 and ERC1-ALK, the latter successfully treated by targeted-therapy. ALK/NTRK fusion partners TPR and KANK1 suggest unusual high-grade morphology/behavior. Clinicoradiologic, morphologic, and phenotypic algorithms can prompt molecular-targeted immunostains or NGS for final classification and promising inhibitor therapy.


Assuntos
Biomarcadores Tumorais/genética , Neoplasias Femorais/genética , Fusão Gênica , Neoplasias de Cabeça e Pescoço/genética , Sarcoma/genética , Neoplasias de Tecidos Moles/genética , Neoplasias Torácicas/genética , Adolescente , Adulto , Idoso , Algoritmos , Antineoplásicos/uso terapêutico , Criança , Pré-Escolar , Extremidades/patologia , Feminino , Neoplasias Femorais/diagnóstico , Neoplasias Femorais/tratamento farmacológico , Neoplasias Femorais/patologia , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias de Cabeça e Pescoço/patologia , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Fenótipo , Prognóstico , Sarcoma/diagnóstico , Sarcoma/tratamento farmacológico , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/patologia , Neoplasias Torácicas/diagnóstico , Neoplasias Torácicas/tratamento farmacológico , Neoplasias Torácicas/patologia , Parede Torácica/patologia , Adulto Jovem
18.
J Investig Med High Impact Case Rep ; 9: 23247096211042236, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34459267

RESUMO

Rhabdomyosarcoma is a malignant soft tissue sarcoma of primitive mesenchymal cells, showing varying degrees of striated skeletal muscle cell differentiation. It is a very common cancer of childhood and adolescence, but rarely seen in the adult population. Here, we present a case of a 33-year-old male presented with a poorly differentiated desmin positive alveolar rhabdomyosarcoma in the left arm. The prognosis of alveolar rhabdomyosarcoma in adults is very poor, frequently detected at advanced stages or with metastases. The alveolar subtype in particular has been found to have a more aggressive course with a high rate of metastasis. Recent studies have shown that using pediatric treatment guidelines resulted in better survival outcomes and local control, but the survival rates are still below that of the pediatric population. Newer studies are looking into using specific molecular markers for more targeted therapy in hopes of further improving survival rates in the adult population.


Assuntos
Carcinoma , Rabdomiossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Adulto , Criança , Humanos , Masculino
19.
Cir Cir ; 89(4): 538-541, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34352867

RESUMO

ANTECEDENTES: El sarcoma de células dendríticas foliculares es una enfermedad rara, caracterizada principalmente en sitios nodales como la cabeza, el cuello y la orofaringe, aunque puede ser extranodal, como en el bazo y el hígado. En la mayoría de los casos cursa asintomática, pero puede presentar síntomas generales, dolor abdominal o fiebre. La inmunohistoquímica es indispensable para llegar a un diagnóstico definitivo. PRESENTACIÓN DEL CASO: Mujer de 40 años, con abultamiento submaxilar en el cuello, región frontoparietooccipital derecha, y sequedad de mucosa oral. Se manejó inicialmente como un síndrome de Sjögren, que fue descartado por el resultado histopatológico de la biopsia de glándula salival. Posteriormente se realizó biopsia de ganglio del cuello, que reportó sarcoma de células dendríticas foliculares con expresión inmunohistoquímica positiva para CD23 y negativa para CD21 y ACL. Se manejó con samario y tuvo una sobrevida de 3 meses desde el diagnóstico. CONCLUSIONES: el sarcoma de células dendríticas foliculares es raro y la sobrevida es corta. BACKGROUND: Follicular dendritic cell sarcoma is a rare pathology, it occurs mainly in nodal sites such as head, neck, oropharynx, although extranodal presentation such as spleen and liver may occur. In most cases it is asymptomatic but may present general symptoms, abdominal pain or fever. Immunohistochemistry is essential to make a definitive diagnosis. CASE PRESENTATION: Forty year-old woman, with submaxillary lesion, in the neck, right fronto-parieto-occipital region with dry oral mucosa. It was initially managed as a Sjögren's syndrome ruled out by the histopathological result of salivary gland biopsy. Subsequently, a neck ganglion biopsy was performed that reported follicular dendritic cell sarcoma, with positive immunohistochemical expression for CD23 and negative for CD21 and LCA. It was managed with samarium with a survival of 3 months from the time of its diagnosis. CONCLUSIONS: Follicular dendritic cell sarcoma is rare and its global survival is short.


Assuntos
Procedimentos Cirúrgicos do Sistema Digestório , Sarcoma , Neoplasias de Tecidos Moles , Adulto , Biópsia , Feminino , Humanos
20.
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
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