1/137. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
2/137. Mesenchymal hamartoma of the liver--new insight into histogenesis.BACKGROUND/PURPOSE: Mesenchymal hamartoma (MH) of the liver is thought to develop from the ductal plates of the prenatal liver. This immunohistochemical study was performed to gain insight into the pathophysiology of its development. methods: Specimens from four MHs with adjacent liver, in one case from a biopsy and from the resected lesion after 6 years follow-up, were investigated with immunostaining on cryostatsectionswith antibodies against cytokeratins, vimentin, desmin and alpha-actin, as well as von willebrand factor (factor viii), fibroblast growth factor (FGF) receptors, FGF-1 (acidic FGF), FGF-2 (basic FGF), and the proliferation-associated Ki67 antigen. RESULTS: Fibrous tissue of MH stained positive not only for vimentin, but also for desmin and alpha-actin, whereas cytokeratins and factor viii showed specific staining in biliary cysts and endothelial cells, respectively. All mesenchymal cells expressed proteins of the FGF receptor family. Although FGF-1 was only scarcely detectable, there was an accumulation of FGF-2 in borderline areas of liver to MH. Multiple Ki67-positive mesenchymal cells could be identified in these regions in all three MHs. However, we could not detect any proliferative activity in the MHs after follow-up. CONCLUSIONS: The proliferative process in MH is still active during early childhood. FGF-2 may have a role in promoting this process. The positivity for desmin and alpha-actin of the lesions suggests that fat-storing (Ito) cells of the immature liver may be involved in the development of MH.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
3/137. Brunner's gland hamartomas: report of three cases.Brunner's gland hamartoma is a rare duodenal tumor, which grew larger than 2 cm in diameter in a very limited number of cases. Since the first description in a patient with fatal duodenal intussusception by Cruveilhier in 1835, approximately 143 cases have been reported in the English literature, and only 25 cases had tumor growth to more than 2 cm in diameter. To the best of our knowledge, only 4 cases have been reported in taiwan. We present three of Brunner's gland hamartomas. In one patient the tumor was located on the secondary portion of the duodenum, which presented with massive tumor bleeding and measured 3.0 cm in diameter. The other two were both located on the duodenal bulb, which presented with abdominal pain and measured 2.0 and 1.3 cm in diameter, respectively. One of the patients received endoscopic ultrasonography which showed specific findings. Two patients received laparotomy and tumor excision; the other one received endoscopic polypectomy. All 3 patients recovered well without any complications.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
4/137. 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 = 512.7056383644keywords = adipose, fat (Clic here for more details about this article) |
5/137. Congenital smooth muscle hamartoma of the conjunctival fornix.PURPOSE: Congenital smooth muscle hamartomas are benign tumors composed of proliferating smooth muscle cells. They are usually seen as abnormal patches of skin. Ocular involvement of congenital smooth muscle hamartomas is unusual, with rare reports of patients with external eyelid involvement or proptosis resulting from orbital tumors. We describe a patient with a congenital smooth muscle hamartoma that involved the tarsal conjunctival fornix. methods: review of the patient's medical records, including the results of ophthalmologic, radiologic, and histologic examinations. RESULTS: A healthy 2-year-old boy was initially seen with a conjunctival mass. He had a discrete, gray, cystic-appearing lesion in the inferior fornix of the left eye. A magnetic resonance imaging study revealed no signs of extension of the lesion into the orbit. The lesion was surgically excised. Histologic sections showed large bundles of smooth muscle with a fibrotic background and interdigitating fat, consistent with a diagnosis of a congenital smooth muscle hamartoma. CONCLUSION: To our knowledge, this is the first report of a patient with a congenital smooth muscle hamartoma arising from the conjunctival fornix. It presumably originated from either the smooth muscle of the vascular endothelium or from the capsulopapebral muscle. Congenital smooth muscle hamartoma should be considered in the differential diagnosis of cystic-appearing conjunctival fornix lesions.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
6/137. Uterine adenolipoleiomyoma: a rare hamartomatous lesion.An apparently unique intramural uterine lesion is described for which we propose the name adenolipoleiomyoma. On gross examination, a well-circumscribed white intramural mass contained focal fatty areas. histology showed the mass to be composed of smooth muscle, adipose tissue, and endometrial, endocervical, and tubal type glands. The endometrial glands were surrounded by typical endometrial stroma. There was no evidence of adenomyosis in the uterus outside the lesion. This is only the second report of such a lesion within the uterus and the first with an intramural location. We believe it to be a benign hamartomatous lesion.- - - - - - - - - - ranking = 512.7056383644keywords = adipose, fat (Clic here for more details about this article) |
7/137. 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 = 511.7056383644keywords = adipose (Clic here for more details about this article) |
8/137. 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 = 511.7056383644keywords = adipose (Clic here for more details about this article) |
9/137. Hyperostotic macrodactyly and lipofibromatous hamartoma of the median nerve associated with carpal tunnel syndrome.A new case with 14-year follow-up of an extremely rare variety of congenital hand macrodactyly is presented. The disease characteristically presents a diffuse proliferation of fibrofatty tissue, but in this special type, osteocartilaginous deposits around the joints can also be found. The case presented included the troublesome feature of a lipofibromatous hamartoma in the median nerve at the wrist and its branches producing carpal tunnel syndrome. The patient obtained benefit from carpal tunnel release and epineurolysis. The hyperostotic development was managed with conservative resection of the periarticular osteochondromas. The literature reviewed suggests that the hyperostotic cases of macrodactyly do not differ from general cases of this congenital condition, except for the osteochondral deposits. These tumours develop during adulthood or after previous trauma, before epiphyseal closure.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
10/137. Muscular hamartoma of the breast: a rare breast lesion containing smooth muscle.hamartoma of the breast is an uncommon entity, usually presenting as a well-demarcated breast mass. Microscopically, the lesion is composed of mammary glandular component, fibrous stroma, adipose tissue, and smooth muscle in variable proportions. Among the variants of breast hamartoma, muscular hamartoma is rare. This lesion should be differentiated from other breast tumors that contain smooth muscle element. We report a breast lesion of a 36-year-old woman diagnosed as a muscular hamartoma in which the muscular component is cellular and some mitotic figures are present. The criteria to distinguish between benign and malignant smooth muscle lesions in the breast, emphasizing mitotic count, are also discussed.- - - - - - - - - - ranking = 511.7056383644keywords = adipose (Clic here for more details about this article) |
| | Next -> |