Cases reported "Glomus Tumor"

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1/22. Congenital multiple plaque-like glomangiomyoma.

    Congenital glomus tumor is a rare clinical variant of glomus tumor, and glomangiomyoma is the least frequent histologic type of glomus tumor. We report a case of congenital multiple plaque-like glomangiomyoma in a 38-year-old man with multiple nodules and plaques on his left arm and forearm. Histopathologic study showed an angiomatous, nonencapsulated tumor with numerous highly folded dilated vascular lumina scattered throughout the dermis. The lumina were lined by a single layer of flat endothelial cells, and one to several rows of glomus cells were observed adjacent to the endothelial cells. Around large vessels, there was a gradual transition from glomus cells to elongated mature smooth muscle cells with thin and long "blunt-ended" nuclei. Immunohistochemically, there were strong positive reactions for cytoplasmic alpha-smooth muscle actin in glomus cells and smooth muscle cells, vimentin in glomus cells and endothelial cells, and desmin in the smooth muscle cells only. To our knowledge, this is the first case report of congenital multiple plaque-like glomus tumor with the microscopic appearance of a glomangiomyoma.
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2/22. Localized multiple glomus tumors of the face and oral mucosa.

    We describe a 54-year-old patient with an unusual localization of localized multiple glomus tumors. She had several nodules on the left mandibular area, lower lip, and anterior part of the buccal mucosa. A biopsy taken from the left mandibular area showed cystically dilated vessels lined by endothelial cells and a few outer layers of glomus cells in the dermis to subcutis. Localized multiple glomus tumors are rare, and both their facial and oral mucocal localization are extremely rare.
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3/22. Malignant glomus tumor in the branchial muscle of a 16-year-old girl.

    Malignant glomus tumor is an extremely rare neoplasm and its histological features are not well characterized. We report a 16-year-old female patient with a malignant glomus tumor. The patient was admitted to our hospital presenting with a mass in the right upper arm that she had noticed for the previous 6 months. Computed tomography and magnetic resonance imaging revealed an expanded mass involving the surrounding tissues. At surgery, an ill-defined and expanded mass was found, 5 x 4 x 3 cm in size, in the right branchial muscle. The tumor was extirpated, along with neighboring muscle tissues. Histologically, tumor cells were round to short-spindle shaped, forming solid sheets admixed with vessels of varying size. Their nuclei were uniformly oval to round, and their cytoplasms were slightly eosinophilic. The growth pattern of the tumor cells resembled that of glomus tumor, but mitotic figures were frequent (as high as 10 per 10 high-power fields). Immunohistochemically, the tumor cells were positive for vimentin and muscle actin, but negative for desmin. There were no areas typical of benign glomus tumor or sarcomatous change. These findings led us to a diagnosis of primary malignant glomus tumor arising de novo. There has been no recurrence or metastasis for 21 months after wide excision.
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4/22. Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature.

    We studied 4 new cases of juxtaglomerular cell tumor and compared their morphologic and immunohistochemicalfeatures with 2 renal hemangiopericytomas and 5 cutaneous glomus tumors. The juxtaglomerular tumors were resectedfrom 2 males and 2 females (mean age at diagnosis, 23 years). Three patients manifested with severe hypertension. Tumors ranged from 2.2 to 8.0 cm and were well circumscribed. The tumors consisted of solid sheets and nodules of variably sized tumor cells with round, oval, and spindled nuclei alternating with edematous microcystic foci. Nuclear atypia, present in all tumors, was a prominent feature in 2. Mitotic activity was not identified. All cases showed hemorrhage, numerous mast cells, and thick-walled blood vessels. Unusual features included coagulative tumor necrosis, a hemangiopericytoma-like vascular pattern, and hyalinized stroma. All tumors were immunoreactive for CD34 and actin. Ultrastructural analysis revealed the presence of rhomboid-shaped renin protogranules. patients were treated by partial or radical nephrectomy and followed up for 14 to 48 months. There were no recurrences or metastases. The characteristic clinical and morphologic features of juxtaglomerular cell tumor permit distinction from renal hemangiopericytoma and other renal tumors.
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5/22. Glomangiomyoma (glomus tumour) of the pancreas: a case report.

    Glomus tumours occur in many sites. We report the first case of a glomangiomyoma in the pancreas of a 17-year-old girl. The tumour was 5cm in diameter and consisted of rounded glomus cells, blood vessels and spindled smooth muscle cells. The glomus and smooth muscle tumour cells showed moderate diffuse cytoplasmic staining with vimentin, muscle-specific actin and smooth muscle actin. There was weak focal positivity for desmin in the spindle cell component only. The patient is alive and well with no evidence of recurrence 24 months after surgery.
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6/22. Pulmonary glomus tumour: a case initially diagnosed as carcinoid tumour.

    Pulmonary glomus tumours are rare lesions, with few cases reported previously. Herein, we present the clinical and pathological features of a case of pulmonary glomus tumour. A 29-year-old female patient presented to our clinic complaining of cough, dyspnoea and left-sided chest pain. Computed tomography (CT) of the thorax revealed a nodular lesion causing obstruction of the left main bronchus. Fibreoptic bronchoscopy demonstrated a polypoid mass occluding the left main bronchus 10 mm distal to the main carina. Bronchoscopic biopsy was interpreted histologically as carcinoid tumour. Bronchotomy plus mass extirpation was performed with left thoracotomy. Microscopically, a tumoral structure composed of uniform cells with a round centrally located nucleolus and narrow eosinophilic cytoplasm was seen. Thin-walled vessels lined with endothelium were interspersed between tumoral structures. The cells were stained chromogranin and cytokeratin negative and strongly vimentin positive. The pathological diagnosis for the thoracotomy specimen was pulmonary glomus tumour. Follow-up chest CT was negative for recurrent tumour and the patient remains free of disease 17 months after surgery.
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7/22. Primary glomangioma of the liver: a case report and review of the literature.

    Glomangiomas are a subset of glomus tumors that have a rich vascular network. Although a majority of the glomus tumors occur in the skin of the hand, they have also been reported in the deep soft tissue, bone, lungs, and gastrointestinal tract, especially the stomach. To our knowledge, only one such case has previously been reported primarily occurring in the liver. We report a case of a glomangioma primarily arising in the liver of a 57-year-old man who presented with right flank pain of several months' duration. A 3.0-cm hepatic mass was excised and consisted of numerous, small-to-medium branched vessels with the stroma containing small, round, regular cells with sharply outlined round-to-oval nuclei. Immunostains showed the tumor cells to be diffusely positive for vimentin and smooth muscle actin and to be focally positive for calponin. collagen IV stained the pericellular matrix. The immunostain for CD34 highlighted the vascular network as well as outlined the tumor cells in many areas. Coexpression of actin and CD34 in glomus tumors, although unusual, has recently been reported in the literature. Despite its bland histology, the large tumor size and deep visceral location were suggestive of aggressive behavior; thus, a close clinical follow-up was recommended. The patient had an unremarkable postoperative course and has no evidence of metastatic disease 12 months after the procedure. An accurate diagnosis and an understanding the biology of this rare disease, especially in an unusual location, are crucial to its management.
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8/22. glomus tumor: a rare location in the upper lip.

    glomus tumor is a benign neoplasm composed of glomocytes and vessels in varying proportions. It appears as a single painful nodule typically found in the dermis and the subcutaneous tissue of the extremities. A rare appearance of the tumor in the upper lip is presented. Differential diagnosis (along with clinical, microscopic, and immunohistochemical findings) is discussed.
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9/22. glomus tumor of the duodenum: a case report.

    We describe a rare case of glomus tumor of the duodenum. The tumor was detected in a 46-year-old man being investigated for episodes of gastrointestinal bleeding. The excised specimen showed a 2.3 x 1.5 x 1.5 cm gray-tan tumor involving the mucosa and the submucosa. The overlying mucosa was focally ulcerated. Histologically, the tumor had a solid pattern of sharply demarcated, round glomus cells with prominent pericytoma-like vessels of varying sizes. The neoplastic cells stained for vimentin, smooth muscle actin, and focally for synaptophysin. These histologic and immunohistochemical features are consistent with a diagnosis of glomus tumor of the duodenum.
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10/22. Primary glomangioma of the esophagus mimicking esophageal papilloma.

    We report a case of glomangioma of the esophagus in a 28-year-old woman who presented with a 3-year history of vague discomfort, pain and heat in the neck. At initial gross examination, the tumor mimicked an esophageal papilloma. The resected esophageal specimen contained a polypoid, whitish-gray mass, which measured 3 cm in maximum diameter. Microscopically the tumor consisted of loose fibrovascular stroma heavily infiltrated with mononuclear inflammatory cells and covered with focally hyperkeratotic, parakeratotic and acanthotic squamous epithelium without atypia. In the deeper area immediately above the true muscular layer of the esophageal wall, microscopical examination revealed the neoplasm consisting of numerous, small-to-medium branched vessels covered by regular endothelium and filled with erythrocytes. The loose stroma around the vessels contained poorly circumscribed nests of small, round to oval cells with a uniform appearance. Immunohistochemically, the tumor cells were immunoreactive for smooth muscle actin and vimentin and non-immunoreactive for CD34, CD117, desmin, pan-cytokeratin, synaptophisin, neuron-specific enolase and S-100 protein. Despite its bland histology, the infiltrative growth pattern was suggestive of aggressive behavior; thus, an appropriate clinical follow-up was recommended. An accurate diagnosis and an understanding of the behavior of these rare tumors, especially in an unusual location, are crucial to their management and clinical outcome.
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