1/73. Recurrent sclerotic fibroma of the skin.The pathologic findings of a sclerotic fibroma of the skin may resemble those of other benign cutaneous lesions. In this report, the sequential pathologic changes of a recurrent sclerotic fibroma of the skin during a period of 7 years are documented. As the lesion became older, there was not only a decrease in cellularity, but also a progressive organization of the collagen bundles toward a "plywood-like" appearance.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/73. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure.A case of scleredema diabeticorum of Buschke associated with nuchal fibroma and organic solvent exposure is reported. The patient presented with a neck mass causing discomfort and restriction of movement. Histological examination showed this to be a nuchal fibroma. Additionally, there was widespread induration of the skin of his trunk which was asymptomatic. A biopsy showed features of scleredema. This is the first reported association of these two conditions, both of which show increased and thickened collagen bundles without significant fibroblast proliferation. They differ by the occurrence of mucin in scleredema, although this is not always demonstrable, particularly in late lesions. The possibility that nuchal fibroma is an end stage, localized form of scleredema is canvassed. The patient's medical history included insulin-dependent diabetes mellitus with complications of retinal vessel thrombosis and peripheral neuropathy. The patient also had significant past exposure to a wide variety of chemicals, including organic solvents.- - - - - - - - - - ranking = 6keywords = organ (Clic here for more details about this article) |
3/73. Benign ureteral tumors. Four case reports and a review of the literature.The diagnosis of benign ureteral polyps should be considered especially in patients younger than 40 years with filling defects of the proximal ureter on the intravenous pyelogram (IVP). The excellent results of conservative organ-saving surgery (open resection, ureteroscopic resection, laser coagulation) require precise diagnostic management preoperatively in these patients.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/73. 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 = 54.460306922155keywords = nerve (Clic here for more details about this article) |
5/73. 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 = 9.0767178203592keywords = nerve (Clic here for more details about this article) |
6/73. 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 = 9.0767178203592keywords = nerve (Clic here for more details about this article) |
7/73. Giant-cell fibroblastoma: a case report emphasising the presence of hyperplastic subplasmalemmal linear densities in continuity with granular matrices in the extracellular space.The histological, immunohistochemical and ultrastructural features of a case of giant-cell fibroblastoma from the soft tissues of the chest wall in a 48-year-old female are described with special reference to the cell surface and matrix. Subplasmalemmal linear densities (SLDs) characterised cell surfaces, and exhibited excessive development of the dense external component: foci of identical dense material were present in the matrix. The nature of these dense foci, both the external component of the SLD and those free in the extracellular space, was investigated by light microscope immunostaining for fibronectin, laminin and collagen IV. All three proteins stained vessels. There was weaker but positive staining for tumour cell surfaces and matrix, consistent with the widely dispersed nature of the dense foci. Given their fine structural appearance, these dense foci can be referred to as granular matrices. Given also that the matrix protein immunostaining pattern is consistent with the distribution of these granular matrices as observed by electron microscopy, they may be provisionally interpreted as a kind of basement-membrane-related granular matrix. The presence of these proteins emphasises the point that, while giant-cell fibroblastoma fibroblasts lack a lamina, they nevertheless bear basement-membrane-related proteins organised, however, in a non-laminate fashion. The observations reinforce the need to qualify immunostaining results by ultrastructural investigation in order to understand the organisation of immuno-detected proteins and are discussed in terms of their diagnostic and possible biological significance.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
8/73. 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 = 9.0767178203592keywords = nerve (Clic here for more details about this article) |
9/73. A case of orbital solitary fibrous tumor.BACKGROUND: Solitary fibrous tumor is a spindle cell neoplasm that most commonly arises in the pleura and very rarely involves the orbit. CASE: A 38-year-old woman presented with slowly progressive proptosis of 3 months duration and optic nerve head edema in her right eye. magnetic resonance imaging revealed a well-circumscribed, round mass lesion, which showed isointensity to the gray matter in a T1-weighted image, and variegated intensity in a T2-weighted image and contact with the optic nerve in her right orbit. The tumor was successfully removed by anterior orbitotomy. OBSERVATIONS: The tumor showed a "patternless pattern" of tumor cell arrangement, alternating hypercellular and hypocellular areas, a hemangiopericytoma-like pattern, and thickened strands of collagen. Immunohistochemically, the tumor cells were positive for CD34 and vimentin, and all were negative for other markers of epithelial, neural, muscular, histiocytic, and vascular endothelial cell elements. The tumor was diagnosed as a solitary fibrous tumor, and the patient was doing well with no evidence of recurrence 15 months after surgery. CONCLUSIONS: This case was the 19th reported case of solitary fibrous tumor in the orbital region. CD34 is a highly sensitive marker for solitary fibrous tumor.- - - - - - - - - - ranking = 18.153435640718keywords = nerve (Clic here for more details about this article) |
10/73. Intrarenal solitary fibrous tumor of the kidney report of a case with emphasis on the differential diagnosis in the wide spectrum of monomorphous spindle cell tumors of the kidney.Solitary fibrous tumor (SFT) is a neoplasm that can occur in the urogenital tract, and is also reported occurring in the spermatic cord, seminal vesicles, urinary bladder, prostate, and kidney. Furthermore, it is most important to consider its existence in the kidney, because it is usually diagnosed as renal cell carcinoma pre-operatively. To our knowledge, only 10 cases of SFT have been reported in the kidney to date. We report the clinico-pathological features of an intrarenal SFT occurring in a 31-year-old woman. The tumor, measuring 8.6 cm in its greatest diameter, completely replaced the cortex and the medulla of the middle region of the right kidney, compressing the pelvis. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumor was composed of a proliferation of bland-looking vimentin , CD34 , bcl2 and CD99 spindle cells exhibiting a haphazard to storiform growth pattern, pushing borders, and a low mitotic rate (2 mitoses x 10 HPF). We placed emphasis on the differential diagnostic problems, i.e., its differentiation from other primary monomorphous benign and malignant spindle cell tumors of the kidney, such as fibroma, benign fibrous histiocytoma, hemangiopericytoma, inflammatory myofibroblastic (pseudo-)tumor, leiomyoma, angiomyolipoma with predominant spindle cell smooth muscle component, benign peripheral nerve sheath tumors, renal mixed epithelial/stromal tumors, adult type mesoblastic nephroma, fibrous type monophasic synovial sarcoma, malignant peripheral nerve sheath tumors, fibrosarcoma, and low-grade fibromyxoid sarcoma.- - - - - - - - - - ranking = 18.153435640718keywords = nerve (Clic here for more details about this article) |
| | Next -> |