Retroperitoneal leiomyosarcoma. A clinicopathologic analysis of 36 cases.

Thirty-six cases of retroperitoneal leiomyosarcoma form the basis for this retrospective clinicopathologic study. This group comprised 24 females (67%) and 12 males (33%), whose ages ranged from 12 to 94 years (median, 60 years.). The clinical presentation, invariably nonspecific, consisted of pain or weight loss, typically associated with a palpable abdominal mass. Of the 30 patients with follow-up data, 23 (77%) died of retroperitoneal leiomyosarcoma. Grossly, the bulky often multinodular tumors, which ranged from 7.5 to 35 cm in maximal dimension (median, 12.8 cm), varied from firm to soft. In addition to the classical microscopic picture of leiomyosarcoma as manifested by interlacing fascicles of slender eosinophilic cells, other less frequently encountered, morphologic variations of malignant smooth-muscle tumors were also observed. Although absolute minimal criteria for a malignant tumor diagnosis could not be established, the findings suggest that a retroperitoneal smooth-muscle tumor that measures at least 7.5 cm in greatest dimension and that has as few as 1 mitosis/10 HPF is capable of metastasis.

Cranial fasciitis of childhood.

Nine cases of fibroblastic lesions occurring in the cranium of young children were reviewed. The age of the patients at the time of initial treatment ranged from three weeks to six years (median 18 months), with the lesions being congenital in two cases. There was 2:1 male predominance. The size of the lesions averaged 2.5 cm in greatest dimension with the largest being 9.0 cm. All cases presented as rapidly growing masses with a preoperative duration of only two months. The lesions presented as soft-tissue masses deep in the scalp with involvement of the underlying cranium in all eight of the cases in which roentgenograms or operative reports were available for review. Characteristically, there was erosion of only the outer table of the skull, although in three cases the lesion extended through the inner table to attach to the underlying dura mater. It was not possible to detect the exact site or origin, although origin from one of the deep fascial layers of the scalp or the underlying periosteum seems most likely. Microscopically, the lesion appeared to be a proliferation of loosely arranged fibroblasts which most closely resembled nodular fasciitis. Mitotic figures as well as foci of osseous metaplasia were present. Treatment consisted of excision of the mass with local resection or curettage of the affected underlying bone in some cases. Followup revealed a benign clinical course with no recurrent or aggressive behavior.