Primary proximal epithelioid sarcoma of the lung successfully treated with pneumonectomy and adjuvant chemotherapy

Abstract

Primary sarcomas of the lung and proximal epithelial sarcomas (PESs) are extremely rare. Inactivation of INI1 has been found in the majority of epithelioid sarcoma (ES). We report the third known case of a primary PES of the lung along with immunohistochemical data. A 41-year-old man with HIV infection, on highly active antiretroviral therapy, presented with haemoptysis, shortness of breath and progressive weight loss for 2 months. He was eventually diagnosed with stage IIA cT2bN0M0 grade-2 primary PES of the lung. This patient underwent pneumonectomy and adjuvant chemotherapy with ifosfamide and doxorubicin. He remains in remission 36 months since diagnosis. Our case stands to help other clinicians as treatment of such rare cases is often reliant on case reports. We also posit a possible pathogenic mechanism given a history of HIV infection in this patient. The association of INI1 mutation with other atypical sarcomas in patients with HIV infection merits further evaluation.

Primary pulmonary epithelioid sarcoma: a case report

“…The first patient underwent radiation therapy and remained in remission for 78 months [ 3 ]. In the second case, pneumonectomy and adjuvant chemotherapy with ifosfamide plus doxorubicin were performed, and the patient remained in remission for 36 months after the diagnosis [ 4 ]. The third patient underwent chemotherapy and radiation therapy and died because of pulmonary metastasis and pneumonia 4 years after the initial treatment [ 5 ].…”

Primary pleural epithelioid sarcoma of the proximal type: a diagnostic and therapeutic challenge

“…Proximal epithelioid sarcoma (PES) usually involves males in their early decades of life (7). PES has been reported frequently in the extremities but other locations including palate, penis, perineum and the scalp have been reported in the literature too (8).…”

“…Histological diagnosis could be challenging at times due to shared histological features with other neoplasms including extrarenal rhabdoid sarcomas, clear cell sarcoma and squamous cell carcinoma. Due to the same histological mimicry ES need to be differentiated from inflammatory conditions like nodular fasciitis and tenosynovitis as well as from granulomatous processes (7).…”

A typical presentation of primary pulmonary epithelioid sarcoma misdiagnosed as non‐small cell lung cancer

“…According to our extensive literature review, all of these cases were proximal type. To the best of our knowledge, this is only the third case of primary pulmonary ES . Primary sarcoma of the lung usually presents as bronchogenic carcinoma, as there are no specific symptoms or CT manifestations .…”