We report on a 58-year-old Caucasian woman that was hospitalized owing to a monstrous ovarian tumor on the right side with a three-month history of sudden abdominal growth and subsequent dyspnea. Sonography and CT scan rendered an additional myomatous uterus with requirement of a bilateral hysterosalpingectomy. The last consultation with a gynecologist had been about eight years prior. The complete staging was without evidence of further neoplasms and the patient was otherwise healthy. The patient had eight deliveries and one abortion. She finally was referred to a dermatologist because of multiple agminated dense erythematous nodules restricted to the skin of the left shank and the right flank with a few aberrant lesions at the dorsal trunk only (Figure 1). The patient reported occasionally associated painful sensations but did not request further treatment. The nodules obviously had rapidly appeared in the context of alvine tuberculosis at the age of 13. However, a relapse of the previous mycobacteriosis was excluded upon punctuation of the ovarian tumor.
Histopathologically, there was evidence of a severe fibroleiomyomatosis of the uterus with an associated dexter ovarian cystadenoma (diameter 25 cm) (Figure 2). A biopsy of the cutaneous lesions on the right flank revealed pilar leiomyomas consisting of a plaque-like confluent fascicular spindle cell proliferation obviously originating in the muscles of hair erection. Nuclei were cigar-shaped and in part exhibited vesicular pseudoinclusions. There was no mitotic activity. However, some plump cells were seen and interpreted as a clue to ancient changes (Figure 3). Smooth muscular differentiation was confirmed immunohistochemically by coexpression of actin, desmin and smooth muscle actin. The proliferation index as detected by nuclear Ki67 expression was less than 1%. Blood samples with addition of EDTA were analyzed molecularpathologically and a complete deletion of the fumarate hydratase gene was detected.