Six cases of locally aggressive and/or potentially malignant glomus tumors are described. On the basis of clinical and pathologic criteria, the following classification is proposed. The first category is a locally infiltrative glomus tumor (LIGT) which has the usual glomus histologic features. The second group is a cytologically malignant tumor arising and merging with a typical glomus tumor, designated glomangiosarcoma arising in a benign glomus (GABG). The third category and the most difficult to recognize is the de novo glomangiosarcoma (GADN), which must be distinguished from other round cell sarcomas. Most of these locally aggressive glomus tumors are vimentin positive and are immunoreactive for muscle-specific actin. Electron microscopic examination in one GABG case showed cells with numerous microfilaments and pinocytotic vesicles; a second GADN case contained cells with microfilaments and an incomplete basal lamina. As a group these locally aggressive or potentially malignant glomus tumors are larger and more deeply located than the conventional glomus tumor. Although 50% of these tumors recurred locally, none have metastasized.
The current report describes the case of a 13-year-old girl with an extralobar pulmonary sequestration who presented with chest and back pains without evidence of infection. Her symptoms were discovered to be secondary to torsion and infarction of the pulmonary sequestration. Although pulmonary sequestration is not an uncommon differential diagnosis for chest masses, it is rare for it to present in this manner.
The presence of well-differentiated rhabdomyoblasts at the end of therapy for rhabdomyosarcoma has been noted. This study was undertaken to investigate the therapeutic implications of the presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma. Six patients with pelvic rhabdomyosarcoma (bladder-prostate, 4; vulvovaginal, 2) with disease diagnosed between the years 1974 and 1992 were sequentially investigated by cystoscopic or vaginoscopic examination and biopsy during and after completing therapy. All six patients received treatment according to prevailing therapeutic protocols. Biopsy material from all six patients at the end of therapy documented the presence of well-differentiated rhabdomyoblasts. Repeated biopsies demonstrated the presence of rhabdomyoblasts; however, they appeared to decrease in number with time. Mitotic activity was not observed in the biopsy materials obtained. All six patients are alive without evidence of disease from 37 to 233 months after therapy ended. The presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma is a common finding. The biologic nature of these well-differentiated rhabdomyoblasts is not completely known, but they do not appear to connote the persistent presence of malignant disease and are not an indication for the continuation of therapy.
We report the first maternal pulmonary adenocarcinoma metastatic to the fetus as well as an updated literature review. Review of the literature revealed that there have been only 67 cases of maternal malignancy metastatic to the products of conception. These were mostly malignant melanoma and hematopoietic tumors. A 46-year old multiparous woman with metastatic pulmonary adenocarcinoma, diagnosed at 23 weeks gestation, delivered a male infant who appeared normal at birth. The mother died 2 days after delivery. The child developed multiple scalp tumors a 2 weeks of age. The tumors recurred rapidly after initial resection. Wide local excision of the involved scalp and skin graft coverage was performed at 14 weeks of age. Histopathology of these tumors was identical to that of the maternal tumor. The maternal origin of these tumors was confirmed by fluorescence in situ hybridization (FISH). The child is now 5 years old and free of disease.
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