1/53. Giant hamartoma of the lung.In the following case of giant pulmonary hamartoma, a 62-year-old woman exhibited a huge tumor shadow in the right lung field, whilst remaining asymptomatic. A thoracotomy revealed a solid intrapulmonary mass histologically diagnosed as a cartilaginous hamartoma with no evidence of malignancy. The tumor was resected by enucleation and there has been no recurrence for 40 months since surgery. Parenchyma-saving enucleation or excision is a safe and sufficient procedure for peripheral hamartomas of any size.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/53. Splenic vascular lesions: unusual features and a review of the literature.hamartoma and peliosis are uncommon splenic lesions. Approximately 120 splenic hamartomas and 40 splenic peliosis have been reported in the English literature. In the present study, a unique case of multiple splenic hamartomas with peliosis was reported. The splenic lesions were incidental findings in a 36-year-old man with ruptured sarcomatoid renal cell carcinoma. They were diagnosed clinically as metastatic renal cell carcinoma. On pathological examination, peliosis was noted in the splenic hamartomas as well as in the splenic parenchyma. In addition, the clinicopathological features of five other splenic hamartomas (including one giant hamartoma) noted in our department were presented. A re-evaluation of the features of the splenic hamartomas documented in the English literature was also done.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/53. 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 = giant (Clic here for more details about this article) |
4/53. A giant vascular hamartoma of the breast in a child.Vascular tumors of the breast are uncommon. Perilobular hemangiomas, the commonest histologic subtype of breast hemangiomas, constitute majority of microscopic, vascular mammary tumors. Macroscopic hemangiomas and vascular hamartomas of the breast are distinctly rare. We describe a giant, vascular hamartoma of the right breast in a five year old girl and discuss pertinent literature.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
5/53. Giant hypothalamic hamartoma with cystic change: report of two cases and review of the literature.We describe the MRI findings in two patients with giant hypothalamic hamartomas with cystic areas. Cystic change within hypothalamic hamartomas is rarely reported in the literature.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
6/53. Desmoplastic neuroepithelial tumor of infancy in the nevus sebaceus syndrome: report of a unique constellation and review of the literature.The nevus sebaceus syndrome (NSS) is a neurocutaneous disorder characterized by unilateral hyperplasia of skin appendages and skeletal hemihypertrophy, hemimegalencephaly, or hemiatrophy along with disabling seizures. Despite the proneness of the dermal stigmata to eventually undergo neoplastic transformation, the malformative lesions of the central nervous system rarely evolve into frank tumors. We present the case of a 10-year-old girl with left-sided sebaceus nevi, ipsilateral enlargement of the skull, and a desmoplastic neuroepithelial tumor (DNET) in the right fronto-parietal area of the brain. The tumor was removed by surgery. Histologically, it corresponded to a mitotically active small-cell anaplastic astrocytoma with genuine desmoplasia. Investigative methods included immunohistochemical positivity for glial fibrillary acidic protein, lack of expression of neuronal markers, and ultrastructural documentation of sheaths of basal lamina and collagen around tumor cells. A survey of the literature of brain tumors associated with NSS revealed two cases of histologically verified pilocytic astrocytomas, and one each of a choroid plexus papilloma, a mixed glioma, and a meningioma, as well as a subependymal giant cell astrocytoma--the latter possibly in an overlap syndrome of NSS and tuberous sclerosis. We hypothesize that the tumor described herein, one involving both atypical differentiation and enhanced growth potential, is paradigmatic of neuropathological events to be expected in the NSS.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/53. Giant hamartoma of the breast.hamartoma of the breast is a rare clinico-pathologic entity. Its clinical diagnosis can be extremely difficult; however, diagnosis is not difficult when it is made on the basis of a combination of radiologic and pathologic features. Its differential diagnosis includes a circumscribed fibrocystic mass, fibroadenoma, lipoma, cystosarcoma phylloides, and various carcinomas. A high index of suspicion is mandatory. In this study, we report a case of giant hamartoma of the breast in a young postlactational female patient, including treatment of the tumor by excisional biopsy and correction of the resulting breast deformity by mastopexy. Clinical, radiologic, and pathologic features are discussed.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
8/53. Giant mammary hamartoma diagnosed by stereomicroscopic analysis of the mammary glandular tree in an adolescent girl: report of a case.This report describes a rare case of a giant hamartoma that developed in the right breast of a 17-year-old girl. No abnormalities were found by endocrinological studies and a well-circumscribed tumor, approximately 20 cm in diameter, was easily enucleated without bleeding during surgery, following which the bilateral breasts became nearly symmetrical. Histologic features revealed predominant fibrous stroma and scattered normal or occasionally dysplastic mammary glands without neoplastic properties. No distorted lobular structures indicating fibroadenoma characteristics were observed. Subgross and stereomicroscopic analysis of serial 2-mm-thick sections revealed mature normal lobules and predominant fibrous interstitial components. Therefore, the tumor was diagnosed as a giant hamartoma of the breast, according to the histologically non-neoplastic features and the delimited macroscopic appearance. This is a rare mammary gland disease characterized by the fact that although each of the histological components seemed to be normal, their constitution was abnormal. It appears that not only histological features but also clinical details are indispensable for the diagnosis of this disease.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
9/53. Giant hamartoma of the ascending colon in an adult with ulcerative colitis.We report an unusual case of giant colonic hamartoma in a 50-yr-old man with ulcerative colitis. The clinical, endoscopic, and histological aspects of this case are discussed. In addition, the possible pathogenesis of colonic hamartoma is reviewed and related to our patient with ulcerative colitis.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
10/53. Neuropathology of tuberous sclerosis.In the cerebrum of patients with tuberous sclerosis (TSC), there are three types of nodular lesions: cortical tubers, subcortical heterotopic nodules and subependymal giant cell astrocytomas. Histologically, these hamartias and hamartomas contain abnormal giant cells that show evidence of abnormal differentiation of immature neural cells. Recent identification of the TSC1 and TSC2 genes has facilitated studies of the molecular pathology of TSC. The expression of their protein products, hamartin and tuberin, is altered in various TSC lesions. However, the molecular mechanism by which cortical tubers develop remains to be elucidated. The Eker rat, a naturally occurring animal model of TSC, will provide a powerful tool for future investigations of TSC.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
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