Diagnostically Challenging Epithelioid Soft Tissue Tumors

James, Aaron W.; Dry, Sarah M.

doi:10.1016/j.path.2015.05.002

Cited by 12 publications

(18 citation statements)

References 110 publications

(110 reference statements)

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“…Clear cell sarcoma of the soft tissue and malignant melanoma may also exhibit similar histological features to PEComa, along with positive immunohistochemical staining of melanocytic markers[ 20 ]. However, clear cell sarcomas of the soft tissue display cellular nests separated by a fibrocollagenous network and strong S100 expression, whereas PEComas typically show a delicate vascular rich stroma and do not express S100.…”

Section: Discussionmentioning

confidence: 99%

“…ASPS is composed of large epithelioid cells with abundant cytoplasm, arranged in nests or sheets that are separated by a delicate vascular network of capillaries[ 21 ]. However, ASPS can be differentiated from PEComa by immunohistochemical staining based on negative staining for melanocytic markers, which aids in differentiating from PEComa[ 20 ]. The differential diagnosis of PEComa from gastrointestinal stromal tumors and dedifferentiated liposarcomas is based on the absence of CD117, DOG-1, MDM2 , and CDK4 expression.…”

Section: Discussionmentioning

confidence: 99%

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TFE3-expressing malignant perivascular epithelioid cell tumor of the mesentery: A case report and review of literature

Kim

Lee

Choi

et al. 2020

WJCC

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BACKGROUND Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor that exhibits an epithelioid and spindle cell morphology. The tumor is characterized by immunoreactivity for melanocytic and myogenic markers but can be misdiagnosed as more common tumors with similar characteristics, including gastrointestinal stroma tumors or leiomyosarcomas. Recently, a subset of PEComas has been reported to harbor a transcription factor binding to TFE3 fusion. Herein, we report a rare case of TFE3 -expressing malignant PEComa arising from the mesentery. CASE SUMMARY A 50-year-old woman presented with abdominal discomfort for 3 months. Results of laboratory tests were all within the normal ranges, and the patient had no notable medical history. Magnetic resonance imaging revealed a large tumor on the right side of the pelvic floor, which was originally suspected to be a primary ovarian tumor. However, during surgery, the tumor was revealed to have originated from the mesentery. Histologically, the tumor was composed of bundles of spindle cells and sheets of epithelioid cells. Extensive coagulative necrosis and numerous mitotic figures were observed. Immunohistochemistry revealed that the tumor cells were positive for smooth muscle actin, HMB-45, and TFE3 expression. Tumor involvement of the rectal serosa was identified, leading to a final diagnosis of malignant PEComa of the mesentery. Surgical resection was followed by adjuvant chemotherapy. No recurrence or metastasis was observed over a 6-month follow-up period. CONCLUSION Malignant PEComa of the mesentery is extremely rare and should be distinguished from morphological mimics through differential diagnosis and immunohistochemistry.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

TFE3-expressing malignant perivascular epithelioid cell tumor of the mesentery: A case report and review of literature

Kim

Lee

Choi

et al. 2020

WJCC

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“…A diverse array of rare soft tissue tumors shows epithelioid cytomorphology [35]. The possibility of metastatic carcinoma and metastatic melanoma must always be excluded before considering a soft tissue tumor in this category, since the former are so much more common.…”

Section: Epithelioid Tumors Of Soft Tissuementioning

confidence: 99%

Limited biopsies of soft tissue tumors: the contemporary role of immunohistochemistry and molecular diagnostics

Hornick

2019

Modern Pathology

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Diagnosing soft tissue tumors is challenging, even on ample incisional biopsies or resection specimens. There are more than 100 distinct types of soft tissue neoplasms, including more than 80 benign and intermediate mesenchymal tumors and around 40 soft tissue sarcomas. Accurate diagnosis relies first upon recognition of characteristic histologic and cytologic features, including architecture, stromal characteristics, vascular patterns, and dominant cytology; these features may not be represented or apparent in limited core needle biopsy or fine needle aspiration specimens. Once a differential diagnosis is established, application of immunohistochemistry and cytogenetic or molecular diagnostic assays (especially fluorescence in situ hybridization) is used in an attempt to reach a specific diagnosis. In recent years, the diagnostic armamentarium for soft tissue tumors has expanded dramatically, following the discovery of molecular alterations that underlie the pathogenesis of soft tissue tumors. These include new diagnostic immunohistochemical markers that serve as useful surrogates for molecular genetic alterations. Availability of such markers has improved our ability to render accurate and specific diagnoses based on limited biopsy samples. In this review, examples of recently developed markers for the diagnosis of selected soft tissue tumor types will be discussed, including solitary fibrous tumor (STAT6), malignant peripheral nerve sheath tumor (H3K27me3), epithelioid hemangioendothelioma (CAMTA1), dedifferentiated liposarcoma (MDM2), and CIC-DUX4 sarcoma (WT1 and ETV4).

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“…The pathological finding displayed a cystic mass with 6.5 cm at the longest diameter, dark red in section and cysts could be found locally. A strong expression of S-100, HMB45 and Vimentin was detected in immunohistochemical staining (Figure 3), which was inclined to the diagnosis of the CCS of soft tissue[2,3].…”

Section: Final Diagnosismentioning

confidence: 99%

“…However, on account of sharing similar morphology with malignant melanoma, SSC seems more likely to derive from neural crest cells, which produces melanin typically and has positive HMB-45 and S-100 mark stained in immunohistochemistry[2]. CCS has been usually reported to locate at distal end of extremities and rarely at trunk[3]. According to the literature review, there is no report of CCS in pleural cavity till now.…”

Section: Introductionmentioning

confidence: 99%

Clear cell sarcoma of soft tissue in pleural cavity: A case report

Chen

Yang

et al. 2019

WJCC

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BACKGROUNDClear cell sarcoma (CCS) of soft tissue is a rare malignant soft tissue sarcoma usually reported to locate at distal end of extremities and rarely at trunk. Herein, we report a case of CCS in pleural cavity.CASE SUMMARYA 31-year-old male was admitted for an uncertain mass in left pleural cavity in routine physical examination without any symptoms. A VATS surgery was performed to remove the tumor. The pathological finding displayed a cystic mass with 6.5 cm at the longest diameter, dark red in section and cysts could be found locally. A strong expression of S-100, HMB45 and Vimentin was detected in immunohistochemical staining, which was inclined to the diagnosis of the CCS of soft tissue. The patient refused chemotherapy, radiotherapy and targeted therapy because of the personal financial situation. Follow-up computed tomography scans were done at the 90th postoperative day and the 180th postoperative day, and no obvious sign of recurrence was found till now.CONCLUSIONCCS of soft tissue also can be found in pleural cavity although in an extremely rare incidence. Radical resection is useful to improve the prognosis.

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Diagnostically Challenging Epithelioid Soft Tissue Tumors

Cited by 12 publications

References 110 publications

TFE3-expressing malignant perivascular epithelioid cell tumor of the mesentery: A case report and review of literature

TFE3-expressing malignant perivascular epithelioid cell tumor of the mesentery: A case report and review of literature

Limited biopsies of soft tissue tumors: the contemporary role of immunohistochemistry and molecular diagnostics

Clear cell sarcoma of soft tissue in pleural cavity: A case report

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