Primary Inferior Vena Cava smooth muscle tumor with diffuse bizarre giant nuclei and low mitotic rate: a nomenclatural conundrum

Abstract A male patient with obstructive jaundice was found to have an incidental nodule within the inferior vena cava (IVC), below the level of the renal vein, on abdominal imaging. At the time of the Whipple's procedure for pancreatic adenocarcinoma, the IVC mass measuring 3.4 x 2.7 x 2.2 cm, was also removed. Histologically, the lesion was well-circumscribed, composed focally of spindle shaped cells with cigar-shaped nuclei reminiscent of smooth muscle, and a dominant pervasive, pleomorphic, bizarre giant cell component. Two mitoses per 10 high power fields were identified in the most mitotically active area of the entire tumor; the vast majority of the tumor being mitotically inert. Additionally, no evidence of coagulative necrosis was noted. The bizarre giant cells had multi- and poly-lobated configurations and several were replete with nuclear pseudo-inclusions. Both the spindle cell and pleomorphic components displayed strong immunoreactivity for all smooth muscle markers. This lesion conformed morphologically to a smooth muscle tumor with bizarre nuclei or so-called symplastic/bizarre leiomyoma, as encountered in the uterus. However, current thinking based on location in the IVC and the presence of any mitotic activity with cellular atypia, makes this lesion a leiomyosarcoma. Perhaps more pragmatic terminology would be smooth muscle tumor with bizarre nuclei and low malignant potential since the limited number of cases described thus far, appear to have a more indolent course.