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  Table of Contents  
LETTER TO THE EDITOR
Year : 2015  |  Volume : 52  |  Issue : 1  |  Page : 97-98
 

Proximal type of epithelioid sarcoma of back with metastasis to humerus at presentation: Indicating aggressive behavior


1 Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, India
2 Department of Orthopedics, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, India
3 Department of Surgery, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, India

Date of Web Publication3-Feb-2016

Correspondence Address:
V A Amrut
Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.175609

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How to cite this article:
Gayatri S P, Amrut V A, Sanjay D D, Shirish S P, Pankaj K. Proximal type of epithelioid sarcoma of back with metastasis to humerus at presentation: Indicating aggressive behavior. Indian J Cancer 2015;52:97-8

How to cite this URL:
Gayatri S P, Amrut V A, Sanjay D D, Shirish S P, Pankaj K. Proximal type of epithelioid sarcoma of back with metastasis to humerus at presentation: Indicating aggressive behavior. Indian J Cancer [serial online] 2015 [cited 2019 Aug 24];52:97-8. Available from: http://www.indianjcancer.com/text.asp?2015/52/1/97/175609


Sir,

Epithelioid sarcoma (ES), first described by Enzinger, is an uncommon soft tissue sarcoma seen in the distal extremities of young adults.[1] It is usually a slow-growing tumor with tendency to recur locally and metastasize, which has been documented in 40-45% cases.[2] This case is uncommon on account of ES presenting as fracture humerus due to metastasis, which prompted us to report this case.

A 67-year-old female presented with pain and swelling in the left arm since 15 days and swelling on lower back. On general examination, the patient was moderately built with good general health. Local examination revealed that there was tenderness and swelling at the upper end of left humerus. Radiological examination revealed pathological fracture of upper end of left humerus. Subcutaneous swelling was evident in the left lower back region, measuring 4.1 × 4.0 × 3.3 cm. On elucidating details of history, patient revealed that back swelling was 3 months prior to swelling at the upper end of humerus. Routine hematological and biochemical parameters including renal and liver function tests were within normal limit.

Incisional bone biopsy of the humerus lesion and excision biopsy of the back mass with wide margins was carried out. The soft tissue mass was well-circumscribed, firm, and grey-white. On cut surface, it was tan-colored, solid, with areas of hemorrhage. Both the biopsies showed similar histopathological and immunohistochemical (IHC) features: The tumor tissue was composed of round to polygonal cells arranged in sheets, separated by irregular fibrous septae. The cells had intense eosinophilic cytoplasm and pleomorphic nuclei with prominent eosinophilic nucleoli. Some of the cells showed rhabdoid appearance [Figure 1] and [Figure 2]. IHC staining showed reactivity for cytokeratin (CK), vimentin, and epithelial membrane antigen (EMA) [Figure 3]. Desmin was focally positive, mainly in the rhabdoid-appearing tumor cells [Figure 4]. The tumor cells were negative for CD 34, smooth muscle actin, HMB-45, and S-100. On this basis, the differential diagnosis of proximal type ES with metastasis to humerus and metastatic carcinoma were considered. Metastatic carcinoma was ruled after detailed clinical and radiological work up. Histopathological and IHC features favored the diagnosis of proximal type ES with metastasis to humerus. Patient is now on follow-up since 6 months with high dose of chemotherapy.
Figure 1: Tumor cells in sheets with pleomorphism, prominent nucleoli, and abundant eosinophilic cytoplasm (H and E, ×100). Insect showing tumor cells with rhabdoid features (H and E, ×400)

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Figure 2: Section from humerus lesion showing pleomorphic tumor cells in between the bony trabeculae. (H and E, ×100)

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Figure 3: Tumor cells showing immunoreactivity for pan CK, EMA, and vimentin and negative for CD 34 (IHC, ×100)

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Figure 4: Desmin positivity in the rhabdoid-appearing tumor cells. (IHC, ×100)

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ES is usually multinodular with a central necrosis surrounded by bland polygonal cells with eosinophilic cytoplasm and peripheral spindling. The classic- type ES has cells with only mild atypia, although they can appear more pleomorphic in recurrences or metastases. Therefore, it has a wide range of differential diagnosis including granuloma annulare, melanoma, and epithelioid vascular neoplasms. Hence, ES is characterized by diagnostic difficulties, both clinically and histopathologically.[3] On this account, the IHC studies are of paramount importance. Epithelioid sarcomas regularly express vimentin, CK, and EMA, and about half of the epithelioid sarcomas are positive for CD34.[4] In the present case, though CD34 was negative, vimentin, CK, EMA and desmin were positive.

An aggressive subtype of ES has been known as the “proximal-type. It is more frequently seen in the pelvis and perineum of young to middle-aged adults. It usually lacks the conventional “granuloma-like appearance.” In addition to presence of oval to polygonal cells, it shows characteristic large cells with'rhabdoid' traits.[5] Desmin expression has been noted in the rhabdoid cell component of proximal type of ES.[5]

In the present case, tumor cells revealed rhabdoid features, but necrosis was absent. At the same time, metastasis was observed in the upper end of humerus, indicating aggressive nature. In a series of 20 cases, it has been observed that 65% of the patients developed local recurrence and 75% of the patients developing metastases, primarily to the lymph nodes, lung, bone, and skin.[6] Total surgical excision with clear margins and high-dose chemo-radiotherapy represent optimum treatment and achieve low rates of local recurrence.[1],[4]

It is important to identify ES from a list of differential diagnoses in view of its aggressive behavior. A timely diagnosis with optimal treatment can be helpful in improving the outcome.

 
  References Top

1.
Enzinger FM. Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer 1970;26:1029-41.  Back to cited text no. 1
[PUBMED]    
2.
Rekhi B, Gorad BD, Chinoy RF. Clinicopathological features with outcomes of a series of conventional and proximal-type epithelioid sarcomas, diagnosed over a period of 10 years at a tertiary cancer hospital in India. Virchows Arch 2008;453:141-53.  Back to cited text no. 2
    
3.
Armah HB, Parwani AV. Epithelioid sarcoma. Arch Pathol Lab Med 2009;133:814-9.  Back to cited text no. 3
    
4.
Fisher C. Epithelioid sarcoma of Enzinger. Adv Anat Pathol 2006;13:114-21.  Back to cited text no. 4
    
5.
Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD. “Proximal-type” epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series. Am J Surg Pathol 1997;21:130-46.  Back to cited text no. 5
    
6.
Hasegawa T, Matsuno Y, Shimoda T, Umeda T, Yokoyama R, Hirohashi S. Proximal-type epithelioid sarcoma: A clinicopathologic study of 20 cases. Mod Pathol 2001;14:655-63.  Back to cited text no. 6
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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