Primary Adrenal Leiomyosarcoma: Case Report and Review of Literature

Abstract

Primary adrenal leiomyosarcomas are rare and usually symptomatic at presentation. The presentation of a large adrenal mass should raise suspicion of adrenal leiomyosarcoma as a differential diagnosis. To our knowledge, primary adrenal leiomyosarcoma has been reported in 20 cases in the English literature. Here we describe a case of primary adrenal leiomyosarcoma in a 76-year-old Caucasian female. The patient complained of right upper quadrant abdominal pain for 2 months. Contrast-enhanced magnetic resonance imaging showed a heterogeneous 10.5 cm adrenal mass with a suspected inferior vena cava tumor thrombus without lymph node enlargement or distant metastasis. The patient underwent a right adrenalectomy, partial resection of the inferior vena cava and reconstruction of the inferior vena cava with a pericardial patch. Histopathologic examination was consistent with leiomyosarcoma. At 3 months postoperatively, a follow-up computed tomography scan of the chest, abdomen and pelvis without intravenous contrast was done that showed multiple bilateral pulmonary metastatic lesions, bilateral hilar and mediastinal lymphadenopathy, liver metastasis, a new mass at the head of the pancreas, and a new mass at the lower pole of the right kidney. The patient was deemed to be unfit for systemic chemotherapy, and was referred to the hospice service for palliative care. The patient died 4 months after surgery.

Primary Adrenal Leiomyosarcoma in an Arab Male: A Rare Case Report with Immunohistochemistry Study

“…The most common malignant primary adrenal mesenchymal tumor is leiomyosarcoma which usually has asymptomatic presentation. Till now, only around 20 cases have been reported around the world [ 2 – 4 ].…”

“…The age of patients ranged from 30 to 73 years with male to female ratio of 3 : 1 and median size of the tumor is 11–25 cm [ 2 , 4 , 5 ]. With the cut-off size 3 cm for benign tumors, for all larger tumors, preoperative imaging and screening should be performed to assess the resectability of the tumor and the possibility of metastasis from clinically occult tumor [ 1 ].…”

“…The patients usually present with abdominal flank pain, mass or symptoms of adjacent organ involvement like IVC obstruction leading to lower limb swelling, venous gangrene, and pulmonary embolism. They can also present with symptoms of metastasis to liver, lung, and bone leading to shortness of breath, jaundice, bone pain, and paresis [ 4 – 6 , 11 ].…”

Primary Leiomyosarcoma of Adrenal Gland with Tissue Eosinophilic Infiltration

“…The prognosis for primary adrenal leiomyosarcoma is generally poor. Among previously reported cases, only two cases were alive without metastasis after more than 15 months follow-up [ 5 - 7 ]. The distinct profile of the patient is high-grade sarcoma with marked tissue eosinophilia and 18 months survival without metastasis.…”

“…Primary adrenal leiomyosarcoma is a very rare malignant mesenchymal tumor with smooth muscle differentiation. Twenty-two cases have been reported in the English [ 5 , 6 ] and Korean [ 7 ] literature. The association of tissue eosinophilia with leiomyosarcoma is very rare, with only one case of uterine leiomyosarcoma reported in the literature [ 4 ].…”