We present six cases of a distinctive soft tissue tumor which occurred in five women and one man. None of the patients had signs of neurofibromatosis. All tumors occurred on the fingers ( n=5) or the thenar eminence of the hand ( n=1). The mean age of the patients was 33 years. The tumors were 1-2.5 cm in diameter (mean size 1.6 cm). Three patients with follow-up were without signs of recurrence or metastasis. Microscopically the lesions were nonencapsulated and featured a multilobular architecture and both myxoid and pseudocystic change. The lobules varied in size and shape and were separated by variably thickened, dense, sclerotic/collagenous septae. The lobules were composed of two components: schwannomatous and perineuriomatous. The schwannomatous component was immunohistochemically S-100 protein positive and CD34 and EMA negative, and the perineuriomatous component had the appearance of retiform perineurioma. The perineurial parts were mostly S-100 protein and CD34 negative and EMA positive. These two components either formed separate nodules or the schwannomatous tissue surrounded the perineurial parts located in the centers of the lobules. We interpreted the lesions as hybrid tumors with features of schwannoma and retiform perineurioma.

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SMARCB1 (INI1) deficient sinonasal carcinoma is a recently recognized entity with wide histomorphologic spectrum. We present a case of this carcinoma that contained, in addition to a "common" morphology, scattered foci of yolk sac tumor differentiation. The tumor occurred in paranasal sinuses in a 44-year-old woman. Immunohistochemically, it was diffusely negative for INI1, whereas an expression of yolk sac tumor markers (α-fetoprotein, glypican-3, CDX2) was limited to the yolk sac tumor component. For comparison with the present case, we performed INI1 immunostaining on a series of 11 gonadal germ cell tumors with yolk sac tumor differentiation. All of these cases showed strong and diffuse expression of INI1, in contrast with the present sinonasal tumor. Our findings expand the morphologic spectrum of SMARCB1 (INI1) deficient sinonasal carcinoma. In addition, we show preliminarily that gonadal germ cell tumors with yolk sac tumor differentiation are not SMARCB1/INI1-deficient.

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Mullerianosis of the urinary bladder is a very rare and morphologically complex tumor-like lesion. It is composed of several types of mullerian-type lesions, including endometriosis, endocervicosis, and endosalpingiosis. This disease occurs in women of the reproductive age group. In the pathogenesis, implantative and metaplastic origins have been suggested, and they are still under discussion. In the present report, a rare case of this lesion in a 41-year-old woman is described. The bladder contained, in addition to typical mullerianosis, glandular cystitis with focal intestinal metaplasia. An interesting and, to our knowledge, hitherto unreported feature was a rare continuity between the glands of mullerianosis and glands of glandular cystitis. The histologic and immunohistochemical findings in the present case support strongly the metaplastic origin of mullerianosis.