Primary Adrenal Leiomyosarcoma: A Case Report and Literature Review

Abstract

Summary:The case presented here illustrates a 75 year old female patient who underwent surgical resection of a right adrenal mass of uncertain nature. The fi nal histological diagnosis was consistent with leiomyosarcoma arising from the adrenal anatomic site. Primary leiomyosarcoma of the adrenal gland is a very rare malignant mesenchymal neoplasm: to our knowledge, this is only the twelfth case reported in literature. We describe the clinical course and a brief review of clinical and histological features, biologic behaviour, diagnostic approaches and therapeutic strategies. Case ReportA 75-year-old white female was admitted to the Department of Urology of our hospital following the ultrasound fi nding of a right adrenal mass of uncertain nature. Her medical history was unremarkable, except for long-term essential arterial hypertension. For a month prior to hospitalization the patient had complained of persistent and progressive epigastric and right loin pain. Physical examination showed only mild abdominal discomfort at palpation. No enlarged lymph nodes were present. Laboratory tests detected no abnormalities. Urinary levels of vanilmandelic acid, catecolamines and plasmatic level of aldosterone were normal.Chest radiograph and electrocardiogram revealed no pathological fi ndings. Upper gastrointestinal endoscopy detected oesophageal hiatal hernia with refl ux oesophagitis, and total-body CT confi rmed the presence of a right adrenal mass of about 5 × 4 cm, suspected to be an adrenal carcinoma, so radical surgery was planned and performed with the resection of a roundish formation from the adrenal anatomic site. Normal adrenal gland was not visible (Fig. 1).Three days after surgery the patient developed a pulmonary embolism: an inferior vena cava thrombosis was identifi ed by angio-CT scan. Pulmonary embolism was identifi ed by angio-CT scan, too. Moreover, an unexplained progressive drop of hemoglobin concentration down to 7.7 g/dl, requiring infusion of two units of packed red cells, was observed. The patient was transferred to our Intensive Care Unit, where she received routine treatment for pulmonary embolism including anticoagulant therapy fi rst with intravenous heparin and then with warfarin.The clinical course was satisfactory, and subsequent Doppler-ultrasonography confi rmed the return to normal of venous fl ow through inferior vena cava. The patient, clinically stable with a good cardiorespiratory compensation, was discharged on postoperative day 18; continuation of anticoagulant therapy and physical rehabilitation were recommended. No oncological treatment was provided after surgery, and no evidence of recurrent disease was observed one year later.Gross pathological examination of the resected tissue showed a roundish, fi brous neoformation of 8 × 4 × 5 cm. A thrombus was detected inside the adrenal vein; this thrombus was not visible on CT performed before surgery. On sectioning of the mass, no adrenal structure was recognizable. Resection margins were free of disease. Histologically, tissue sect...

Primary Adrenal Leiomyosarcoma: Case Report and Review of Literature

“…Twenty cases have been reported in the English literature since 1981. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] Typically, the disease presents as a symptomatic retroperitoneal mass more than 10 cm in diameter. It can be associated with a mass effect on surrounding organs, involvement of the inferior vena cava (IVC) by a tumor thrombus, local organ invasion or distant metastasis.…”

Primary adrenal leiomyosarcoma: A case report with immunohistochemical study and review of literature

“…[3] A review of the literature revealed only twelve previously reported cases of PAL. [2] The reported age of patients ranged from 30 to 75 years with approximately equal frequency in males and females. All these tumors ranged in size from 11 to 25 cm in greatest dimension.…”

“…[1] Reviewing the literature, we encountered only twelve previously reported cases of primary adrenal leiomyosarcoma (PAL). [2] We believe that this is the thirteenth case of PAL. It has a wide range of differential diagnosis including MPNST, malignant melanoma, gastrointestinal stromal tumour (GIST); metastatic carcinoma and others.…”

Recurrent Primaty Adrenal Leiopyosarcoma: A Complete Literature Review and Presentation of a Rare Adrenal Tumor

“…Radical surgical resection of the tumor is the main goal of therapy. Some authors advocate postsurgical adjuvant radiation therapy for locally advanced malignant disease (10,11). In a recent report, Murart et al (14) suggested that radical surgical resection without adjuvant therapies might be an alternative approach for low-grade leiomyosarcomas.…”