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Introduction: Soft tissue sarcomas constitute a heterogenous category of soft tissue neoplasia composed mostly of uncommon tumours of diverse histology, different biology and varied outcomes. Majority of sarcomas are presented as a painless mass of variable duration (1). Materials and methods: It’s a cross-sectional study where six cases of soft tissue sarcomas were taken which were diagnosed on histopathology and further ancillary techniques like immunohistochemistry were done. The clinical and radiological findings of the cases are as follows. Results: The neoplasm arising from tunica media of inferior venecava. The tumour cells are arranged in interlacing fascicles. The tumour cells show oval to elongated spindle shaped nuceli, moderate nuclear atypia, coarse chromatin, consipicuous nuceloli and moderate eosinophilic fibrillar cytoplasm. Conclusion: With tumours arising in these unusual sites, definitive recognition becomes difficult and requires confirmation by immunohistochemistry and molecular genetic techniques. Molecular pathology has identified key, pathognomonic genetic markers in soft tissue sarcoma, improving diagnostic accuracy and speed.
Soft tissue sarcomas constitute a heterogenous category of soft tissue neoplasia composed mostly of uncommon tumours of diverse histology, different biology and varied outcomes.
Majority of sarcomas are presented as a painless mass of variable duration(1).
Sarcomas like extraskeletal ewings sarcoma, epithelioid sarcoma, synovial sarcoma and epithelioid angiosarcoma have got varied presentation.The Ewing sarcoma family of tumours(ESFT) includes Ewing sarcoma, PNET, and Askin tumour, all sharing a pathognomonic EWSR1 translocation. They appear as sheets of small round blue cells and must be differentiated from lymphoma, rhabdomyosarcoma, neuroblastoma, and other small round cell tumours.2
Extraskeletal Ewing sarcoma (EES) is a relatively uncommon primary tumor of the soft tissues, which accounts for 20-30%.3 Epthelioid sarcoma is a rare malignant soft tissue tumour with predominance in male.4Synovial sarcoma seen in adolescents and young adults. Males are effected slightly more then the females(1.2:1). Most common location is lower extremities.5 Epithelioid angiosarcoma is a distinctive but uncommon tumor, representing the malignant end of the spectrum of epithelioid vascular neoplasms. they constitute less than 1% of all sarcomas. Intraabdominal leiomyosarcoma arising in retroperitoneum, mesentry or omentum usually presents in 5th to 6th decade with preponderance in females.6
Objectives: We present six cases of soft tissue sarcomas which are as follows, extraskeletal ewings sarcoma, epithelioid sarcoma, synovial sarcoma, epithelioid angiosarcoma and leiomyosarcoma diagnosed at Aster whitefield hospital, bengaluru.
It’s a cross-sectional study where six cases of soft tissue sarcomas were taken which were diagnosed on histopathology and further ancillary techniques like immunohistochemistry were done. The clinical and radiological findings of the cases are as follows. Case1) Female patient aged 60 years, clinically presented with complains of pain abdomen and on MRI abdomen pelvis found to have solid cystic lesion in the pelvis of about 11 x 8 x 8 cm. Case 2) The male patient aged 35 years presented with non-healing ulcer on the sole of left foot. Case 3) & 4) Two cases with different location were studied. First case in a 63 years female patient presented with complaints of cough and fever. CECT thorax shows irregular lobulated lesion in posterior segment of right upper lobe, with few calcific foci. Second case presented as a renal mass in the female patient aged 37 years. Case 5) The male patient of age 50 years presented with left thigh swelling and multiple skin nodules over the lesion were seen. Case 6) A female patient complaints of swelling in right lower back. On USG large well defined hypoechoic mass lesion 12.7 x 8 x 11 cm in the inferior aspect of right kidney. CECT showed a large well defined homogenously enhancing solid mass lesion measuring 14.3 x 10 x 11.3 cm.
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Cases |
Age/gender |
Location |
Gross findings |
Histopathological features |
Immunohistochemistry |
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1)Extraskeletal ewings sarcoma |
59 years/female |
Left pelvic masssigmoid colon |
Left pelvic mass: Encapsulated tumour with cut surface of tumour showing predominantly solid areas with focal cystic areas. Sloid areas are soft, grey white to fleshy with areas of necrosis and hemorrhage. |
Tumour cells in sheets, trabeculae, macrofollicular.Focal areas show pseudorosette like arrangement ,lo9.
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CD99, NKX2.2 and FLI-1 are positive. |
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2)Epithelioid sarcoma |
34years/male |
Left foot |
The sole skin is ulcerated. Cut surface, the tumour measuring 4 x 4x 2.6 cm. Grey-white and firm. |
Tumour cells arranged in sheets and lobules separated by thick bands of fibrous tissue and hyalinised stroma. Individual cells are epithelioid in shape and show marked nuclear pleomorphism with vesicular chromatin and prominent nucleoli. |
EMA, PanCK and CD34 are positive. Loss of INI1/SMARCB1 |
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3)Synovial sarcoma |
63 years/female |
3)Right upper lobe lung
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Well circumscribed tumour but unencapsulated measuring 4.1 x 4.1 x 3 cm. Cut surface of tumour is lobulated firm and grey white in colour |
Biphasic tumour composed of spindle cells and gland like epithelial structures. The Tumour cells are arranged in sheets, nests and papillary pattern. The outer lining cells are cuboidal. Underlying areas show monotonous stromal cells with round to oval nuclei, fine chromatin, conspicuous nucleoli. |
TLE1, EMA, CD99, TTF1 are positive. PANCK shows focally poistive. |
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37 years/female |
4) Right renal mass |
Slide and block review |
The tumour is cellular and cells arranged in long intersecting fascicles. The tumour cells are spindle shaped having elongated hyperchromatic nucleus with pointed to truncated ends with moderate eosinophilic cytoplasm. |
TLE1,EMA, CD99 are positive. PanCk is focally positive. NKX2.2, PAX7 PAX8 CAIX are negative |
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5)Epithelioid angiosarcoma |
49years/male |
Left thigh |
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Multilobulated neoplasm. The tumour cells are arranged in solid sheets, nests, alveolar, angiomatous and focal papillary patterns. The tumour cells are epithelioid with round vesicular nuclei exhibiting moderate nuclear atypia, prominent nucleoli and abundant eosinophilic cytoplasm. |
CD31, CD34, ERG are positive. Ki67 is 70%. HMB45 is negative |
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6)Leiomyosarcoma |
46 years/female |
Retroperitoneum, inferior venecava |
The tumour is arising from the wall of inferior venecava. Intratumour part of vein shows compression of lumen by tumour. Cut surface of tumour is grey white and trabeculated |
The neoplasm arising from tunica media of inferior venecava. The tumour cells are arranged in interlacing fascicles. The tumour cells show oval to elongated spindle shaped nuceli, moderate nuclear atypia, coarse chromatin, consipicuous nuceloli and moderate eosinophilic fibrillar cytoplasm. |
Desmin, h-caldesmon, SMA are positive. Ki67 is 40%. SOX10, MDM2, p16, TLE1 are negative.
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Case 1. Extraskelatal ewings sarcoma- Left pelvic mass & sigmoid colon
Figure 1: H&E Of Extraskeletal Ewings Sarcoma.
Figure 2: CD99- Shows cytoplasmic positivity for tumour cells.
Figure 3: NKX2.2 shows nuclear positivity of tumour cells.
Case 2: Epitheliod sarcoma- Left foot
Figure: 4. H & E -Epithelioid sarcoma- Left foot
Figure: 5. H & E-Epithelioid sarcoma
Figure: 6. INI1/SMARCB1 loss in tumour cells.
Figure:7. CD34, Membrane staining of tumour cells.
3) & 4) Synovial sarcoma: Presented both right renal mass & Right upper lobe lung
Figure: 8. H&E Right Renal mass-synovial sarcoma
Figure: 9. H & E Synovial sarcoma
Figure: 10. TLE1-Nuclear positivity of tumour cells
Figure 11. CD99- Membrane positivity of tumour cells.
Figure: 12. H & E -Epithelioid angiosarcoma
Figure: 13. H & E. Epithelioid angiosarcoma
Figure:14. ERG showing nuclear positivity for tumour cells.
Figure: 15. H & E Leiomyosarcoma-inferior venecava wall.
Figure: 16. H & E Leiomyosarcoma-inferior venecava wall.
Figure: 17. H caldesmon showing positive for tumour cells.
Figure: 18. Ki 67 showing 30-40% positive for tumour cells.
Extraskeletal ewings sarcoma with a diagnostic specificity is enhanced by demonstration of diffuse nuclear positivity of NKX2.2.1 The diagnosis of ESFT is routinely confirmed by diffuse membranous CD99 positivity, nuclear FLI1 positivity, and negative LCA. NKX2.2 is a highly sensitive but only moderately specific marker for ESFT.2 Epitheloid sarcoma is most commonly seen in the fingers, hands and forearm. Histology alone is not sufficient for diagnosis. INI1/SMARCB1 loss is the most reliable marker for epithelioid sarcoma, and a CK/EMA/CD34/INI1 panel improves diagnostic accuracy.3,4 Synovial sarcoma also seen as hemangiopericytoma like pattern and often mislabelled. Synovial sarcoma is classified as monophasic, biphasic, or poorly differentiated, and shows EMA, AE1/AE3, BCL2, CD99, and TLE1 positivity.5 Diagnosis on histomorphology of epithelioid angiosarcoma is by the identification of intracytoplasmic lumina, vasoformative areas and positive for endothelial marker(ERG & CD31). Leiomyosarcoma tumours tend to reach a very large size and wide excision is impossible. Desmin is positive in about 70-80% cases and h caldesmon in 60 to 65 % cases.6
With tumours arising in these unusual sites, definitive recognition becomes difficult and requires confirmation by immunohistochemistry and molecular genetic techniques. Molecular pathology has identified key, pathognomonic genetic markers in soft tissue sarcoma, improving diagnostic accuracy and speed.
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