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Primary leiomyosarcoma of the adrenal gland: A review and update of literature.

Anthony Kodzo-Grey Venyo

Primary leiomyosarcoma of the adrenal gland (PLAG) is an uncommon non adrenal hormone producing malignant tumour of smooth muscle origin. PLAG has been sporadically reported more often in the female in comparison with in the male and hence PLAG would tend to be more commonly encountered in females in comparison with males. PLAG can also affect the younger age group contemporaneously with acquired immunodeficiency syndrome and Epstein-Barr virus infection. Patients who have PLAG may present with pain/discomfort in the loin or flank and or upper abdomen or as an incidental finding of an abdominal mass that has been present for many months and getting bigger. PLAG may also present with inferior vena cava syndrome when the tumour has invaded and obstructed the inferior vena emanating in the development of bilateral oedema of the lower limbs and angiomata on the abdominal wall. Basic or routine blood haematology and biochemistry test results tend to be normal as well as blood and urine adrenal hormone levels fall within normal range indicating a non-hormone secreting tumour. Radiology imaging investigations that are utilized to illustrate the adrenal gland position of the tumour and no other lesion in the abdomen include: ultrasound scan of abdomen and pelvis, CT scan of abdomen and pelvis, and MRI scan of abdomen and pelvis. If there is suspicion of obstruction of the IVC then inferior vena cavogram could be undertaken. Diagnosis can be obtained from histology and immunohistochemistry examination of biopsy and or adrenalectomy specimens which in most cases of the classical PLAG show: intersecting and sharply margined fascicles of spindled-cell; characteristic long and blunt ended tumour cell; cytoplasm of the tumours cells that vary from brightly eosinophilic fibrillar to pale cytoplasm; hyperchromatic nuclei; and generally pleomorphism. Positive immunohistochemistry staining for Smooth Muscle Actin (SMA), Desmin, H-caldesmon; Immunohistochemistry studies of primary leiomyosarcoma of the adrenal gland tend to show focally positive staining for: CD34, EMA, Keratin, and S100. With regard to PLAG in Immunocompromised patients, immunohistochemistry studies of the tumour do show that Desmin is less expressed. The main treatment for localized PLAG has been surgery (Adrenalectomy plus excision of adjacent involved organs and if there is tumour in the IVC up to the atrium the tumour is removed). Advanced and metastatic PLAG tumours have been treated by surgery and chemotherapy. The short-term and medium-term outcome of localized PLAG has been good; nevertheless, the long- term outcome is not well documented. With regard to advanced PLAGs, despite surgical removal of the primary tumour and chemotherapy the prognosis has not been good in that the patients generally do not tend to have good medium-term and long-term survival outcomes. In conclusion PLAG is an uncommon tumor which when diagnosed as a localized disease tends to be associated with good initial outcome but advanced PLAGs tend to be associated with inferior outcome. It would appear that the establishment of a global multi-centre trial of various chemotherapy treatment options that would identify treatment options that would improve the prognosis of advanced PLAG is needed.