1/35. 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 = nerve (Clic here for more details about this article) |
2/35. 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 = 0.16666666666667keywords = nerve (Clic here for more details about this article) |
3/35. 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 = 0.16666666666667keywords = nerve (Clic here for more details about this article) |
4/35. 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 = 0.16666666666667keywords = nerve (Clic here for more details about this article) |
5/35. 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 = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
6/35. life-threatening giant mediastinal goiter: a surgical challenge.Mediastinal goiter is a well known benign disease, usually resectable through a cervical approach with minimal morbidity and mortality. Only occasionally a median sternotomy or a lateral thoracotomy may be required. The present case is worthy of presentation because of the exceptional dimension of the disease and the surgical challenge that it presented. In a 72-year-old woman a large intrathoracic goiter of the right thorax caused a severe dyspnoea due to an important contralateral mediastinal shift with compression of the lung, superior vena cava system and trachea. At surgical exploration, through a cervico-sternotomic approach, the mediastinal structures dislocation and the strong adherences between the anomalous neovascularized capsula of the mass and the surrounding structures, complicated the surgical dissection. An accidental lesion of the innominate artery required its reimplantation on the ascending aorta. An immense mass, was finally removed and pathological examination revealed a rare case of neovascularized, pseudosarcomatoid capsula among a benign hyperplastic proliferation. In spite of its benign nature, a giant goiter caused a life-threatening compression of the respiratory tract and lung parenchyma in this patient. The dimension of the lesions, the mediastinal anatomy alterations and the severe intraoperative haemorrhage represented major technical difficulties during surgical resection.- - - - - - - - - - ranking = 0.1364171459537keywords = median (Clic here for more details about this article) |
7/35. 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 = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
8/35. Solitary fibrous tumor with malignant potential arising in sublingual gland.A rare case is described of a solitary fibrous tumor (SFT) with malignant potential arising in the sublingual gland. A 59-year-old man presented with a 4-month history of a slowly enlarging painless mass in the center of the floor of the mouth. The tumor was a well-demarcated, firm mass with a multicystic lesion. The tumor exhibited highly cellular areas of spindle cells with patternless architecture alternating with hypocellular areas. The tumor cells were positive for CD34 and bcl-2 as well as vimentin, and negative for epithelial, myogenic, neurogenic and histiocytic markers. The tumor cells formed multiple satellite nodules around dilated ducts in the multicystic lesion, indicating infiltrative growth. In addition, areas exhibiting higher cellularity with increased mitoses were noticed in the satellite nodules, although cellular atypia was not obvious. These findings led to a final diagnosis of SFT with malignant potential. There has been no recurrence or metastasis for 27 months after the surgery. Solitary fibrous tumor of the salivary gland must be differentiated from various spindle cell neoplasms including myogenic, peripheral nerve sheath, fibroblastic and fibro-histiocytic spindle cell neoplasms, hemangiopericytoma and myoepithelioma. In addition to characteristic morphological features, an immunohistochemical positivity for CD34 and bcl-2 may aid in the diagnosis of SFT.- - - - - - - - - - ranking = 0.16666666666667keywords = nerve (Clic here for more details about this article) |
9/35. Solitary fibrous tumor of the hypoglossal nerve.Summary: We report a case of solitary fibrous tumor (SFT) causing isolated hypoglossal nerve palsy. The neuroimaging appearance of the tumor was indistinguishable from that of schwannoma or meningioma. Immunohistochemical tests demonstrated strong reactivity for CD34 but an absence of staining for S100 and epithelial membrane antigen; this profile is indicative of an SFT. SFTs are mesenchymal tumors that can affect the dura-covered segments of cranial nerves. They may be considered in the differential diagnosis of an isolated cranial nerve palsy.- - - - - - - - - - ranking = 1.1666666666667keywords = nerve (Clic here for more details about this article) |
10/35. Neural fibrolipoma of the foot.Neural fibrolipoma is a benign tumor comprised of hypertrophied fibrofatty tissue with intermixed nerve tissue. We present the case of a neural fibrolipoma of the foot that underwent above-ankle amputation and review the characteristic features of this unique form of localized gigantism.- - - - - - - - - - ranking = 0.16666666666667keywords = nerve (Clic here for more details about this article) |
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