|Ahead of print publication
An Unusual Case of Proximal Variant of Epithelioid Sarcoma of the Shoulder
Raja Rao Nudurupati, Sri Sailaja Rani Mattaparti, Tarun Kumar Suvvari, Divya Bala A M R Salibindla, Mani Kruthika Mantha
Department of General Surgery, Rangaraya Medical College, Kakinada, India
|Date of Submission||13-Jan-2021|
|Date of Acceptance||08-Mar-2021|
|Date of Web Publication||14-Jun-2021|
Raja Rao Nudurupati,
Department of General Surgery, Rangaraya Medical College, Kakinada
Source of Support: None, Conflict of Interest: None
Epithelioid sarcoma (ES) is a rarely occurring malignant soft tissue tumor of uncertain histogenesis. It is most common among young males and commonly seen on the forearm, hand, and fingers. ES begins as a small lump or ulcer, and most were painless. ES has a high rate of recurrence and can spread to other parts of the body easily. Hence, physicians should take the necessary steps to ensure proper diagnosis and treatment. We present an interesting case of a 26-year-old male diagnosed with a proximal variant of ES of the shoulder.
Keywords: Biopsy, chemotherapy, epithelioid sarcoma, shoulder, ulcer
|How to cite this URL:|
Nudurupati RR, Rani Mattaparti SS, Suvvari TK, A M R Salibindla DB, Mantha MK. An Unusual Case of Proximal Variant of Epithelioid Sarcoma of the Shoulder. J Radiat Cancer Res [Epub ahead of print] [cited 2021 Sep 22]. Available from: https://www.journalrcr.org/preprintarticle.asp?id=318401
| Introduction|| |
A rarely occurring malignant soft tissue tumor, epithelioid sarcoma (ES), typically presents in the hands and forearms of young men. The tumor appears as a flesh-colored, firm indolent nodule, or slightly elevated skin lesion. Known for involving the regional lymph node and local recurrence, the tumor may be mistaken for chronic inflammatory processes, as well as fibrohistiocytic tumors and necrotizing granulomas. Misdiagnosis is a major issue, as it can result in improper or delayed treatment, which impacts the chances of patient survival. The nonpainful, indolent growth patterns mean that ES is often present for an extended period before medical attention is sought. The recommended treatment for ES involves wide surgical excision, often in tandem with radiotherapy.,
| Case Report|| |
A 26-year-old male patient came with the complaint of swelling over his right shoulder for 6 months. At first, the ulcer was small but gradually progressed to the present size of 5 cm × 8 cm approximately. The patient had no history of pain or trauma, no history of tingling sensations, and no history of difficulty in limb movements.
On examination, a well-defined 5 cm × 8 cm ulcerated swelling is seen over the posterior part of the right shoulder [Figure 1]. The ulcer has regular margins, sloping edges with slough over the floor, mobile base, and not bleeding on touch. Swelling is nontender, and there was no local rise of temperature, firm to hard in consistency, and mobile. All systematic examination was normal.
|Figure 1: Ulcerated swelling of 5 cm × 8 cm on the posterior part of the right shoulder|
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The patient was taken up for a wide local excisional biopsy. On microscopic appearance [Figure 2], [Figure 3], [Figure 4], the epidermis shows stratified squamous epithelium with focal ulceration, the superficial dermis is showing necrosis and inflammatory infiltrates, and in the deep dermis, there is a poorly circumscribed lesion composed of sheets, islands and discretely placed round to oval cells with moderate eosinophilic cytoplasm, some with cyto-vacuolations, round to oval vesicular nuclei with dispersed chromatin exhibiting pleomorphism. Some spindle-shaped cells with similar nuclear morphology along with binucleated and multinucleated giant cells were observed. Histopathological examination (HPE) of the excisional biopsy was ES with positivity for pan-cytokeratin (CK), vimentin, and epithelial membrane antigen (EMA) and negative for leukocytic common antigen, CK5/6, tumor protein p63 (P63), and S-100 with free margins.
|Figure 2: Large cells with vesicular pleomorphic nuclei, prominent nucleoli, and focal rhabdoid features in the proximal type epithelioid sarcoma|
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|Figure 3: Polygonal or epithelioid cells with minimal pleomorphism and abundant deeply eosinophilic cytoplasm|
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|Figure 4: Multinodular growth pattern with characteristic central necrosis|
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We have done wide excision with surgical margins of 2 cm with frozen section negative. The patient had given external beam radiation therapy for the right shoulder. Chemotherapy (doxorubicin and ifosfamide) has been used to improve survivorship of patient. The patient was followed up for every 3 months, till now, two visits were completed. No recurrence was noted and the patient was asked for a routine follow-up.
| Discussion|| |
ES is a rare, slow-growing malignancy representing the most common primary soft tissue sarcomas of the upper limb. Most common sites involving are hand, fingers, and forearms. It is most commonly seen in young adults of the mean age of 20–39 years. The gender ratio of male: female is approximately 1.8:1 involving distal upper extremity (60%) with a high rate of recurrence and metastasis. Epithelioid can be distal or proximal; the proximal variant is less common and less aggressive. Most tumors present as a firm to hard palpable mass either in deep tissues or dermis; the superficial lesions often ulcerate, causing mistaken diagnosis. Deep tumors mimic ganglion cysts or are attached to tendon sheaths and may be mistaken for giant cell tumors. Definitive diagnosis is made only on tissue biopsy with immunohistochemical (IHC) markers. Magnetic resonance imaging, biopsy, and pathological diagnosis are the most preferred confirmatory diagnosis methods. It helps determine anatomic boundaries and helps differentiate recurrence from postoperative changes after multiple excisions., The differential diagnosis for an ES includes, atypical melanoma, extrarenal rhabdoid tumors, angiosarcoma, epithelioid type of malignant peripheral nerve sheath tumor, synovial sarcoma, rhabdomyosarcoma, and undifferentiated carcinoma.,
IHC studies should show positive for vimentin, CK AE1/AE3, and EMA and negative for S-100 which is typical (distinguishing it from malignant peripheral nerve sheath tumor), endothelial markers (distinguishing it from epithelioid angiosarcoma), CK5/6 (distinguishing it from squamous cell carcinoma), CD34 is expressed in 50%–60% of ESs but is negative in carcinomas helping to distinguish between ES and epithelioid angiosarcoma. P63 is also a useful marker observed in squamous cell carcinomas but absent in ESs. Epithelioid type of malignant peripheral nerve sheath tumor, angiosarcoma, synovial sarcoma, rhabdomyosarcoma, and undifferentiated carcinoma was the differential diagnosis for an epithelioid soft tissue tumor.,,
Wide surgical resection is the most recommended treatment modality for ES. Amputation should be preferred if there are multiple recurrences or if there is not a significant loss of function. Radiation therapy was preferred in some institutions for primary and recurrent cases for limb salvage, with favorable results compared to amputation. Chemotherapy (doxorubicin) has been used for multifocal, large (>5 cm), or metastatic disease. Prognostic factors are younger age at presentation, female gender, smaller size (<2 cm) site at distal extremities, disease stage, and low mitotic index.,,
| Conclusion|| |
ES at the shoulder region was very rare and commonly mistaken due to its similarity to inflammation or ulcer. Clinical behavior and prognosis of the sarcomas were very poor, and the patient should be properly diagnosed and treated. Early detection, wide surgical excision, appropriate HPE with regular follow-up of the patient can lead to a good prognosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]