Case report of an adult with rhabdomyosarcoma of lung with ovarian metastases or the other way around: Do's and don'ts with review of literature.

Abstract

Abstract: Primary pulmonary rhabdomyosarcoma (RMS) is extremely rare (0.07%) in persons aged >45 years and is frequently misdiagnosed as small-cell lung cancer. Little information is available regarding the management of patients, with available literature only limited to a few case reports. We report a rare case of RMS of the lung with multiple metastases with an extensive literature review on the subject. A 52-year-old woman presented with shortness of breath, orthopnea, and chest pain for one and a half months. She also developed abdominal distension in the last month. Imaging showed a large, ill-defined solid cystic mass arising from the retroperitoneum abutting the urinary bladder with multiple adjacent small nodular deposits in serosa and peritoneum with gross ascites along with two large mass lesions in the lower lobe of the left lung and bilateral lung parenchymal metastatic deposits. The biopsy of the adnexal and lung lesions was performed along with immunohistochemistry, which was suggestive of rhabdomyosarcoma. The patient was planned for palliative chemotherapy with biweekly vincristin, adriamycin, cyclophosphamide/ifosphamide, etoposide (VAC/IE) regimen with prophylactic granulocyte-colony stimulating factor support followed by response assessment after 3-4 cycles. However, dose-compressed VAC/IE is highly myelosuppressive and lead to febrile neutropenia in our patient and thus culminated in her death. Hence, the Indian patients should be challenged with dose-compressed VAC/IE with extreme caution. Three-weekly IE/VAC regimen is an alternative.