A rare case report of an aggressive malignant spiradenoma chest wall masquerading as carcinoma of unknown origin - Course of events and review of literature

Abstract

Malignant eccrine spiradenomas are exceedingly rare and aggressive tumours normally arising in long-standing benign eccrine spiradenomas. We present a case of malignant eccrine spiradenoma (MES) that was initially misdiagnosed as carcinoma of unknown origin in spite of extensive workup to look for primary and presented with a large exophytic tumour on the right side of his chest wall, which had fungated. Radiological imaging of the chest region revealed extensive involvement of the local tissues. Patient underwent wide local excision with a 1 cm margin along with right-sided axillary lymph node dissection. Histopathological findings were consistent with MES with tumour deposits in multiple lymph nodes in the axilla. Our case report shows MES is a rare and aggressive cancer, which is difficult to diagnose, highly aggressive with propensity to involve the lymph nodes. May present with local recurrence and also develop distant metastases significantly compromise the quality of life of the patient. It requires the surgeons to have a high index of suspicion for its initial diagnosis, and a multidisciplinary management with surgery, radiotherapy, and chemotherapy is required for complete treatment and needs close follow up.

Keywords

• Chemotherapy
• malignant eccrine spiradenoma
• radiotherapy
• wide local excision