1/66. birt-hogg-dube syndrome and Hornstein-Knickenberg syndrome are the same. Different sectioning technique as the cause of different histology.The autosomal dominant inherited syndromes of Hornstein and Knickenberg (HKS), and Birt, Hogg and Dube (BHDS) are both characterized clinically by the overall spread of multiple flesh coloured papules of the skin. However, it is a matter of debate if colonic neoplasms (adenomas as well as adenocarcinomas) are associated findings in the HKS or rather in the BHDS. Furthermore, histological differences are said to exist between the skin lesions in the two syndromes: whereas perifollicular fibromas were described in the HKS, fibrofolliculomas and trichodiscomas were found in the BHDS. In the present study, we report on a father and his daughter in whom we initially diagnosed a BHDS. We then examined a greater number of the papular lesions in histologic sections cut vertically as well as horizontally to the epidermis. Our results indicate that the histologic differences between the skin lesions in the two syndromes are artificial ones, caused by interpretation of different sectioning planes, and that consequently HKS and BHDS are the same. Therefore, it is necessary to look for colonic polyps in the syndrome in question, regardless if one prefers the name HKS or BHDS for it.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
2/66. fibroma of tendon sheath of the infrapatellar fat pad.We report on a 13-year-old boy who was found to have a fibroma of the tendon sheath associated with the patellar tendon and within Hoffa's fat pad of the knee. This benign tumor has never been described in this location previously. The MRI characteristics are correlated with the histologic findings.- - - - - - - - - - ranking = 5keywords = fat (Clic here for more details about this article) |
3/66. Elastofibroma of the neck.Elastofibromas are benign lesions of the chest wall. We describe the first reported case of elastofibroma in the neck. Imaging features as well as location of the lesion were atypical. On computed tomography and magnetic resonance imaging the lesion contained a marked preponderance of fat, because the lesion arose within fat.- - - - - - - - - - ranking = 2keywords = fat (Clic here for more details about this article) |
4/66. Pedunculated lipofibroma.Pedunculated lipofibroma is a relatively rare form of nevus lipomatosus superficialis. The lesions are large, slow growing, pedunculated tumors. Histologically, groups and strands of fat cells are found embedded among the collagen bundles of the dermis. We presented a woman diagnosed with a pedunculated lipofibroma clinically and histopathologically.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
5/66. Lipofibromatous hamartoma and related peripheral nerve lesions.Three unusual cases of a rare, tumor-like condition, lipofibromatous hamartoma, are reported. This lesion is composed of fibrous and fatty tissue that infiltrates peripheral nerves, typically on the volar aspect of the upper extremities of children and young adults. All three patients had a painless soft tissue mass of the wrist and/or hand, which followed nerve distribution, and only one patient had neurologic symptoms due to compression. diagnosis was made by open biopsy and histologic examination.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
6/66. Elastofibroma of the foot: uncommon presentation: a case report and review of the literature.We report the clinical, microscopic and ultrastructural features of an elastofibroma arising in the foot. The lesion typically occurs in the elderly, and in 85% of cases arises from the connective tissue of the posterior chest wall. The histopathologic features of this lesion are distinctive, and are characterized by a haphazard array of eosinophilic collagen and elastic fibers, associated with fibroblasts and aggregates of mature fat cells. There are only two reported cases in the literature arising in the foot.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
7/66. Fibrous connective tissue lesion mimicking a vestibular schwannoma: case report.OBJECTIVE AND IMPORTANCE: cerebellopontine angle fibromas are rare pathological entities that can mimic the presentation of vestibular schwannomas (VSs). diagnosis of these benign lesions, however, is important, because treatment options may be different. The clinical, radiological, and intraoperative features of these unusual lesions of the cerebellopontine angle are discussed, with review of the relevant literature. CLINICAL PRESENTATION: A 41-year-old man presented with recurrent episodes of diminished hearing on the left side, accompanied by facial ticks and pain on the same side. magnetic resonance imaging and computed tomographic scans revealed a 1.5-cm, primarily intracanalicular lesion, suggesting a left VS. INTERVENTION: The lesion was partially removed through a retrosigmoid suboccipital craniotomy. Its intraoperative appearance and hard fibrotic consistency differed from the classic features of VSs. The pathological findings indicated nontumoral fibrous connective tissue. The lesion exhibited no features of inflammation or fat and was also negative for S-100 staining. Follow-up magnetic resonance imaging scans demonstrated a small residual lesion, which exhibited shrinkage in subsequent magnetic resonance imaging studies. The painful ticks disappeared and facial nerve weakness improved postoperatively. CONCLUSION: Although cerebellopontine angle fibromas may present similar radiological features, their clinical presentation may be somewhat different from that of typical VSs. If a fibroma is suspected, radiosurgery should be avoided; limited surgery may be considered as an option for patients experiencing symptoms. Because fibromas may be intraoperatively noted to be fibrotic and vascular, radical removal may not be easy or justified. After the final diagnosis has been reached, conservative treatment of the residual lesion may be the best option.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
8/66. Nuchal-type fibroma in two related patients with Gardner's syndrome.Nuchal-type fibroma is a distinct subcutaneous and dermal fibrous tissue proliferation that has been previously definitely identified in one patient with Gardner's syndrome and has been possibly present in two others. Gardner's syndrome is an autosomal-dominant condition with variable expressivity that comprises epidermoid cysts, fibrous tumors, osteomas, intestinal polyposis, as well as other findings. We report two cases of nuchal-type fibroma presenting in a 13-year-old boy in the right upper back and in his 60-year-old grandfather in the upper chest at the posterior axillary line. Both individuals carried a diagnosis of Gardner's syndrome and neither of them had diabetes. Although the boy has as of now only presented with cutaneous manifestations of Gardner's syndrome, his grandfather has exhibited both cutaneous and intestinal evidence of this syndrome. In addition, the boy's mother and her sister have documented Gardner's syndrome. light microscopic findings of nuchal-type fibroma from both patients include paucicellular, haphazardly arranged collagen bundles with entrapped adipose tissue. A marked diminution of elastic fibers was noted with Van-Gieson stains. The lesions were diffusely positive for CD34 and contained a few factor xiiia-positive cells. Electron microscopic analysis revealed no differences between the collagen comprising the nuchal-type fibroma as compared with control dermal collagen obtained from skin away from the tumor. These cases strengthen the view that there is an association between nuchal-type fibroma and Gardner's syndrome.- - - - - - - - - - ranking = 68.825629586522keywords = adipose, fat (Clic here for more details about this article) |
9/66. Non-nuchal-type fibroma associated with Gardner's syndrome. A hitherto-unreported mesenchymal tumor different from fibromatosis and nuchal-type fibroma.We describe a unique benign mesenchymal tumor in paraspinal location in a 13-year-old patient with Gardner's syndrome. The Gardner's syndrome in this patient consisted of multiple (more than 100) polyps throughout the entire colon with most in the cecum and rectum, three osteomas in the frontal area of the skull and one in the third right rib, and multiple superficial skin tumors. One of these cutaneous tumors was excised and histologically diagnosed as an epidermal cyst. Both father and uncle of this patient suffered from Gardner's syndrome as well. Microscopically the mesenchymal tumor was histologically different from nuchal type fibroma and fibromatosis. It consisted of a diffusely-growing fibrous mass composed of dense collagenous fibers and relatively numerous, bland-looking, spindle-shaped cells. The collagen fibers had haphazard spacing with no lobular arrangement. The collagen fibers were of a very coarse quality. No entrapment of adipose tissue, skeletal muscle or peripheral nerves was seen in the lesion. Immunohistochemically the tumor was vimentin positive and smooth muscle actin, muscle-specific actin, S-100 protein, cytokeratin and desmin negative.- - - - - - - - - - ranking = 67.825629586522keywords = adipose, fat (Clic here for more details about this article) |
10/66. Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected gardner syndrome and risk for fibromatosis.gardner syndrome (GS), caused by mutations in the adenomatous polyposis coli (APC) gene, is characterized by polyposis coli, osteomas, and various soft-tissue tumors. If undetected or untreated, virtually all patients develop colonic carcinoma at a young age. Early detection, while essential, can be difficult because of attenuated phenotypes or spontaneous mutations. We present the clinicopathologic features of 11 identical fibromatous lesions that we have termed Gardner-associated fibroma (GAF), which not only appear to be a part of the spectrum of lesions associated with GS but, in some cases, represent the sentinel event leading to its detection. The GAFs occurred in 11 patients (5 boys and 6 girls; age range, 3 months-14 years), were solitary (n = 7) or multiple (n = 4), and occurred in the superficial and deep soft tissues of the paraspinal region (n = 7), back (n = 3), face (n = 2), scalp (n = 2), chest wall (n = 2), thigh (n = 1), neck (n = 1), and flank (n = 1). Histologically, GAFs resemble nuchal-type fibromas (NFs), consisting of thick, haphazardly arranged collagen bundles between which are found occasional bland fibroblasts, and having margins that frequently engulf surrounding structures including adjacent fat, muscle and nerves. After surgical excision, four patients developed recurrences that were classic desmoid fibromatoses (DFs). In one patient with multiple GAFs, one lesion had the features of GAF and DF in the absence of surgical trauma. A family history of GS or polyposis (n = 6) or DF (n = 1) was known at the time of surgery in seven patients. In three patients, the diagnosis of GAF resulted in the diagnosis of unsuspected APC in older family members, with the detection of an occult colonic adenocarcinoma in one parent. In the family of the remaining patient, no stigmata of GS were present. Genetic analysis of this child was performed to investigate the presence of a spontaneous (new) mutation; however, no abnormalities were detected. The significance of GAF is that it serves as a sentinel event for identifying GS kindreds, including those with a high risk for the development of DF, and it may potentially identify children with spontaneous mutations of the APC gene. Because NFs and GAFs resemble one another, we suggest that a subset of NF occurring in multiple sites, unusual locations, or children may be GAF.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
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