1/55. fibroma of a tendon-sheath presenting as toe deformity. We describe a rare manifestation fibroma in a tendon-sheath in an 83-year-old man. The patient complained initially of a slowly progressive spreading, apart of the right second and third toes. A mass was found in the plantar aspect of the foot. It was completely excised at surgery. Histological examination revealed sparse spindle or stellate cells with slit-like vessels in the dense collagenous matrix. There had been no recurrence at follow-up 17 months after surgery. ( info) |
2/55. Chondromyxoid fibroma of the nasal bone with extension into the frontal and ethmoidal sinuses: report of one case and a review of the literature. Chondromyxoid fibroma is a rare benign tumor that usually occurs in the long bones. A 50-year-old patient presented with chondromyxoid fibroma of the nasal bone with extension into the frontal and ethmoidal sinuses. This is the fourth case reported to date in the literature. The clinical manifestations of the tumor were very limited, and the appearance at rhinoscopy was misleading. Radiologic imaging showed a soft tissue lesion invading the adjacent bony structures and the dura mater. Surgery was performed by a combined team of otorhinolaryngologists and neurosurgeons, and total excision of the tumor was achieved. The histologic diagnosis of this tumor is difficult because of its similarities to chondrosarcoma. [editorial comment: The authors concisely review management of this rare tumor, emphasizing that complete surgical excision, rather than curettage, is required for long term control.] ( info) |
3/55. Granulocytic sarcoma of humerus, an unusual association of acute myeloblastic leukemia--a case report. Ganulocytic sarcoma (Chloroma) is a tumour of rare variety usually in assocoiation with granulocytic leukemia. It is related to soft tissue with extramedullay infiltration. We present a case of granulocytic sarcoma of humerus which preceded the initial clinical manifestation of acute myeloid leukemia in a young patient which ultimately proved to be FABM2 variety. Though many tissues are affected by this tumour but the most favoured site is the bone. ( info) |
4/55. Papular elastorrhexis in childhood improved by intralesional injections of triamcinolone. Papular elastorrhexis is a rare disease developing asymptomatic skin-colored small papules in adolescence with histopathological loss of elastic fibers. There has been no established treatment for this disease. A 4-year-old Korean boy had multiple, hard, whitish papules on his chest and back for one year. Histopathologic examination revealed focal loss of elastic fibers in the dermis, and X-ray examination showed no bony abnormalities. His skin lesions were improved by intralesional injections of triamcinolone but recurred after four months. ( info) |
5/55. A cell line with multinucleated giant cell formation established from a human giant cell tumor of tendon sheath--preliminary report. We first established a cell line with unique giant cell formation properties from a human giant cell tumor of tendon sheath (GCTTS) arising in the right ankle of a 7-year-old girl. The specimen for cell culture taken from the tumor was heterotransplanted into the back of a BALB/c (nu/nu) nude mouse. An in-vitro cell line was established from a tumor that grew after this heterotransplantation. Only mononuclear cells were observed in the primary culture, and these remained constant in growth. Multinucleated giant cells appeared at passage 3 and were constantly observed thereafter. The fusion of mononuclear cells into giant cells was verified by light and phase-contrast microscopy. This cell line was confirmed to be derived from a human by karyotype analysis and dna fingerprinting. The cell-doubling time was 150 h. This cell line should be useful for studies of the mechanism of multinucleation in giant cell tumors. ( info) |
6/55. Giant gastrointestinal stromal tumor, associated with esophageal hiatus hernia. An 85-year-old woman was admitted to our hospital because of vomiting. An upper gastrointestinal series what showed a large esophageal hiatus hernia, suggesting an association with extrinsic pressure in the middle portion of the stomach. An upper gastrointestinal endoscopic examination showed severe esophagitis and a prominent narrowing in the middle portion of the stomach, however, it showed normal gastric mucosa findings. CT and MRI revealed a large tumor extending from the region of the lower chest to the upper abdomen. From these findings, the tumor was diagnosed as gastrointestinal stromal tumor (GIST), which arose from the gastric wall and complicated with an esophageal hiatus hernia. We performed a laparotomy, however, the tumor showed severe invasion to the circumferential organs. Therefore, we abandoned the excision of the tumor. Histologically, the tumor was composed of spindle shaped cells with marked nuclear atypia and prominent mitosis. The tumor cells were strongly positive for CD34 and c-kit by immunohistochemical examination. From these findings, the tumor was definitely diagnosed as a malignant GIST. As palliative treatment, we implanted a self-expandable metallic stent in the narrow segment of the stomach. The patient could eat solid food and was discharged. In the treatment of esophageal hiatus hernia, the rare association of GIST should be considered. ( info) |
7/55. Collision tumor of the stomach: a case report of mixed gastrointestinal stromal tumor and adenocarcinoma. Collision tumors of the stomach are uncommon. To the best of our knowledge, this is the first case report of gastric collision tumor composed of gastrointestinal stromal tumor (GIST) intermixed with primary adenocarcinoma in the English literature. The adenocarcinoma was determined to be the primary tumor based on histologic features. The tumor cells of the GIST were diffusely and strongly positive for CD34 and CD117, weakly positive for smooth muscle actin (5% of cells), and negative for desmin, S-100 protein, synaptophysin, and cytokeratin. There was no transition between the different components. We hypothesized that the stomach was influenced by the same unknown carcinogen, resulting in a simultaneous proliferation of different cell lines (epithelial and stromal cell). This case represents an example of two independent tumors in a unique one-on-another pattern, namely growth of adenocarcinoma on GIST. ( info) |
8/55. Familial generalized multiple glomangiomyoma: report of a new family, with immunohistochemical and ultrastructural studies and review of the literature. Glomus tumors are benign lesions which often appear as solitary bluish nodules. They can also be multiple, and can be either acquired or congenital. Histopathologically glomus tumors are classified into three different variants: solid glomus tumor, glomangioma, and glomangiomyoma, which is the least frequent type. We report three instances of familial generalized multiple glomangiomyoma in a woman and her two children. Seven members of the family in two consecutive generations were affected, suggesting autosomal dominant inheritance. We performed a thorough clinical study, complete blood and platelet counts, stool for occult blood, karyotype, abdominal echography (which showed an asymptomatic solitary hepatic vascular lesion in one of the patients), gastrointestinal endoscopy, and skin biopsy with immunohistologic and ultrastructural studies. We believe that the terms glomangioma and glomangiomyoma actually designate the same lesion, with transitional areas from typical glomus cells to well-defined conventional smooth muscle cells. Although complete evaluation should be performed in all patients with multiple glomus tumors in order to detect possible occult systemic lesions, we only recommend treatment for symptomatic lesions. To our knowledge, this is the first report of an instance of familial multiple glomangiomyoma. ( info) |
9/55. Elastofibroma dorsi. Report of five cases and review of the literature. Five cases of elastofibroma dorsi are described. The location of the lesions (bilateral in one case) was typical, in the thoracoscapular region. In the first case the diagnosis was established by biopsy; in the remaining cases the diagnosis was suspected based on clinical and magnetic resonance imaging findings and was confirmed by histopathological study after surgical excision. Although these lesions are benign, histological study is advisable to establish a differential diagnosis with malignant neoplastic processes. ( info) |
10/55. Sclerosing stromal tumor of the ovary in a premenarchal female. Sclerosing stromal tumor (SST) is a rare benign ovarian neoplasm of stromal origin with less than 100 cases reported in the literature. Unlike the other stromal tumors, thecomas and fibromas, which tend to occur in the fifth and sixth decades, sclerosing stromal tumors predominantly affect females in the second and third decades. Computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound findings have been described, but have not been reported previously in the pediatric literature. We present a case of SST of the ovary in a 10-year-old premenarchal female, the youngest patient to our knowledge reported in the literature, and describe the ultrasound and CT findings with pathologic correlation. ( info) |