Medical Director of Cancer Rehabilitation Weill Cornell Medicine Jamaica, New York, United States
Case Diagnosis: Angiomyomatous Hamartoma
Case Description: A 42-year-old male with no prior medical history presented at our outpatient clinic with a ten-year history of right thigh swelling that progressed from the size of a golf-ball to a watermelon. He was evaluated by various physicians with blood work unrevealing of abnormalities and ultrasound without evidence of deep vein thrombosis; no additional imaging was pursued. Examination revealed non-pitting edema, skin tightening, large flaking skin folds, and positive stemmer’s sign. Our management included obtaining a CT abdomen and pelvis which revealed adenopathy in right inguinal and iliac lymph nodes with significant lymphedema. He was referred to oncology given concern for indolent lymphoma. PET-CT demonstrated right inguinal and femoral hypermetabolic lymphadenopathy and inguinal lymph node biopsy revealed angiomyomatous hamartoma. He ultimately underwent excision of the tumor. Given the extent of lymphedema, he was referred to lymphedema therapy for complete decongestive therapy.
Discussions: Lymphedema is limb swelling from lymphatic fluid accumulation due to imbalance in the rate of lymph production and lymph removal. Severity of lymphedema is graded on a scale from I to III. Treatment includes complete decongestive therapy with manual lymphatic drainage, compression garments, and decongestive exercises which can reduce edema by 40-60%. The majority of lymphedema is secondary in nature with cases secondary to cancer relatively common. Angiomyomatous hamartoma is an extremely rare and benign vascular tumor with only a handful of cases in the literature. These tumors affect the inguinal lymph nodes and consist of fibrous tissue, irregular blood vessels, and adipocytes. Diagnosis is made by histology and other imaging. Definitive treatment involves complete excision of the lesion.
Conclusions: Angiomyomatous hamartoma is a rare, benign vascular tumor that should be considered on the differential for benign and malignant lymph node tumors which may present as lymphedema.