1/193. Nasal chondromesenchymal hamartoma of infancy: the first Japanese case report.The first Japanese case of nasal chondromesenchymal hamartoma, a rare infantile nasal neoplasm, is presented. A 4-month-old Japanese boy was referred to our Centre because of intranasal mass and oculomotor disturbance. Radiological images showed the intranasal tumor extending to the paranasal sinus, orbit, and anterior frontal fossa. Subtotal resection and radiation therapy to residual tumor were performed. There has been no recurrence of the tumor for 13 years. Histologically, the lesion demonstrated admixture of various mesenchymal elements, including cellular spindle cell stroma with occasional myxoid change, nodules of mature/immature cartilaginous tissue, focal osteoclast-like giant cells, and erythrocyte-filled spaces resembling aneurysmal bone cyst. The histology was consistent with the findings presented by McDermott et al. 1986. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin. chondrocytes in the mature cartilaginous tissue were positive for S-100 and vimentin; chondrocytes in the immature cartilaginous tissue were positive for S-100, vimentin, and smooth muscle actin. Ultrastructurally, the spindle cells showed features of either fibroblast or myofibroblast.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/193. A case of familial angiolipomatosis with Lisch nodules.Familial angiolipomatosis is a rare syndrome that may be confused clinically with neurofibromatosis type 1. This condition is most often inherited in an autosomal recessive manner; however, several reports have been published suggesting an autosomal dominant mode of inheritance. Angiolipomatosis, although somewhat disfiguring, is a benign condition with no known association with malignant neoplasms. This is in contradistinction to neurofibromatosis, an autosomal dominant syndrome associated with a myriad of benign and malignant neoplasms. It is, therefore, important to discriminate this entity from neurofibromatosis when a patient presents with multiple subcutaneous tumors and a family history of similar lesions. Described is a case of a prison inmate with a history of seizures and "neurofibromatosis" without clinical documentation. Lisch nodules were noted on the irides. Postmortem examination showed multiple subcutaneous yellow tumors on the chest and arms. Fine-needle aspiration of 1 mass yielded adipose tissue with prominent vessels; histologic sections of another mass showed angiolipoma. The remainder of the autopsy showed significant coronary artery disease and a remote cerebral infarction of the temporal lobe but no signs of neurofibromatosis. We feel that the presence of multiple angiolipomas in combination with Lisch nodules lends credence to the proposed relationship between fatty tumors and neurofibromatosis suggested by other authors.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
3/193. Fibrous hamartoma of infancy.Fibrous harmartoma of infancy is a benign soft tissue tumor that occurs in the first few years of life. Although the lesion is not distinctive clinically, it has a characteristic microscopic appearance. Only 12 cases of fibrous hamartoma of infancy have been reported in the dermatology literature.- - - - - - - - - - ranking = 0.054950034678082keywords = soft (Clic here for more details about this article) |
4/193. Spiradenoma arising in a nevus sebaceus of Jadassohn: case report and literature review.nevus sebaceus (NS) of Jadassohn is usually a verrucous plaque on the scalp or face that arises secondary to disordered development of epithelial, pilar, sebaceous, and apocrine structures. The emergence of neoplasia is a late stage in the natural history of NS. Although most neoplastic proliferations are benign, several malignant tumors have arisen in this lesion. We describe the first case of a benign spiradenoma arising in an NS on the scalp in a 72-year-old Caucasian woman. Reexcision was recommended to prevent the development of a second neoplastic process and to avoid the rare occurrence of a malignant transformation of the existing neoplasia. The patient declined reexcision and remains under observation. The spectrum of tumors arising in NS are described and are categorized according to behavior. Syringocystadenoma papilliferum is the most commonly observed benign growth, whereas basal cell carcinoma is the most frequently seen malignant process. The signs of tumor development (benign or malignant) within an NS are reviewed, and treatment recommendations are provided. The clinical course of rare and unique aggressive neoplasms originating in NS is summarized.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/193. Polypoid hamartoma of Brunner's gland of the duodenum.BACKGROUND/AIMS: Brunner's gland adenoma is an extremely rare but important entity. Controversy exists over its etiology and pathogenesis, but the present view is that it is a duodenal hamartoma with a predominance of Brunner's gland elements. methods: A case of a 76-year-old woman with a reddish pedunculated polyp prolapsing between the bulb and the second part of the duodenum seen at endoscopy and removed surgically through a longitudinal duodenotomy is presented. RESULTS: The cut surface of the tumor had a grayish color, revealing multiple cystic spaces which on microscopic examination proved to be enlarged Brunner's glands. The hyperplastic glands formed lobules which were surrounded by bundles of fibromuscular and connective tissue. In the adjacent duodenum, large numbers of lobules of well-differentiated Brunner's glands with mucus-secreting epithelial cells were seen. CONCLUSION: The reported case supports the theory that Brunner's gland adenomas are duodenal hamartomas with a predominance of Brunner's gland elements and further shows that a continuity exists in Brunner's glands of the tumor and those of the adjacent duodenum. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 1.5132841056083keywords = connective (Clic here for more details about this article) |
6/193. Trichoblastoma and sebaceoma in nevus sebaceus.We report two cases of an unusual combined adnexal neoplasm arising in a nevus sebaceus (NS). Clinically, both neoplasms presented in two women (46 and 78 years) as single, partially ulcerated nodules within NS situated on the scalp. Histopathologically, each neoplasm showed distinctive aggregations of basaloid cells with features of trichoblastoma adjacent to aggregations of neoplastic cells exhibiting features of sebaceoma. In both cases, typical features of NS were present. To the best of our knowledge, this unusual combined adnexal neoplasm comprised of trichoblastoma and sebaceoma could not be assigned to any previously described histopathologic entity. This "complex" adnexal neoplasm should be distinguished histopathologically from basal cell carcinoma with sebaceous differentiation and trichoblastoma with sebaceous differentiation.- - - - - - - - - - ranking = 5keywords = neoplasm (Clic here for more details about this article) |
7/193. Cutaneous hamartoma of the hand: MR imaging findings.We report two cases of magnetic resonance imaging of the cutaneous hamartoma on the hand, which is a rare benign soft tissue tumor.- - - - - - - - - - ranking = 0.054950034678082keywords = soft (Clic here for more details about this article) |
8/193. Fine needle aspiration cytology of mesenchymal hamartoma of the liver. A case report.BACKGROUND: Mesenchymal hamartoma (MH) of the liver constitutes the third or fourth most common tumor of the liver in childhood and occurs most commonly in the first two years of life. MHs of the liver are seldom aspirated, and reports on the role of fine needle aspiration (FNA) in the diagnosis of MH are scarce. Clinically, cytologically and even histologically, MH can be mistaken for a number of reactive and neoplastic hepatic lesions that may occur in children under 2 years of age. CASE: A 10-month-old Pakistani female presented with a history of a right-sided, nonpainful abdominal swelling. Abdominal computed tomography showed a large, partly solid and partly cystic, heterogeneous hepatic mass. FNA cytology showed clusters of both epithelial and mesenchymal/spindle-shaped cells with pieces of loose connective tissue. A cytologic differential diagnosis of mesenchymal hepatic hamartoma and hepatoblastoma of the possible mixed mesenchymal/epithelial subtype was rendered. The histopathologic diagnosis of the resected tumor mass was benign mesenchymal hamartoma of the liver. CONCLUSION: In children under 2 years of age who present with partly solid and partly cystic hepatic masses, the possibility of MH of the liver should be considered. FNA has a role in the diagnosis of MH. The cytopathologist should be aware of the patient's age, radiologic features and cytologic appearances of this rare, benign neoplasm. Histologic examination of tru-cut biopsies and immunohistochemical stains can help to exclude other pediatric neoplasms that may show cytologic features similar to or mimicking those of MH.- - - - - - - - - - ranking = 3.5132841056083keywords = connective, neoplasm (Clic here for more details about this article) |
9/193. Ectopic hamartomatous thymoma: a case study and review of the literature.Ectopic hamartomatous thymoma is a rare and distinctive tumor found in the deep soft tissues of the neck, which is characterized histopathologically by a mixture of spindle, epithelial, and adipose cell elements. We present a case of this lesion occurring in a 39-year-old male. The characteristic histochemical and immunohistochemical findings of these tumors are demonstrated. In addition, review of the reported histological, immunohistochemical and ultrastructural findings is presented together with a table of clinical findings in the tumors so far described. A brief discussion of theories of histogenesis and possible differential diagnosis is included.- - - - - - - - - - ranking = 0.054950034678082keywords = soft (Clic here for more details about this article) |
10/193. Mammary hamartoma: immunohistochemical study of two adenolipomas and one variant with cartilage, smooth muscle and myoepithelial proliferation.Three cases of mammary hamartoma were investigated immunohistochemically and are described. Case 1 was a 42 year old woman with an elastic hard tumor, 1.5 cm in diameter, in her left breast. Case 2 was a 49 year old woman with a semisoft tumor, 5 x 2 cm, in her right breast. Case 3 was a 47 year old woman with a hard tumor, 5 cm in diameter, in her left breast. In each case, mammography and ultrasonography revealed a benign-looking, well-circumscribed mass without calcification. Histologically, the tumors were composed of adipose tissue, mammary glands, and fibrous and/or fibromuscular tissue. The tumor in case 3 also contained small islands of hyaline cartilage. Immunohistochemical analysis was performed, and epithelial and mesenchymal components were discretely and differentially immunostained except that the smooth muscle component seemed to be derived from myoepithelial cells. Cartilage formation might be the result of metaplasia, and 'metaplastic variant of the mammary hamartoma' or 'choristoma' may be an appropriate term for cartilage-containing mammary hamartoma. Using proliferating cell nuclear antigen (PCNA)-immunostaining, we observed that each component of the tumors had an individual growth rate. This finding may reflect one aspect of the biological characteristics of hamartoma.- - - - - - - - - - ranking = 0.054950034678082keywords = soft (Clic here for more details about this article) |
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