Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.
Adolescent ; Artifacts ; Biopsy ; Ear Canal ; Ear, Middle ; Frozen Sections ; Glomus Jugulare ; Glomus Tumor ; Glomus Tympanicum ; Granulation Tissue ; Humans ; Male ; Paraganglioma ; Paraganglioma, Extra-Adrenal ; Synaptophysin

Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.
Adolescent ; Artifacts ; Biopsy ; Ear Canal ; Ear, Middle ; Frozen Sections ; Glomus Jugulare ; Glomus Tumor ; Glomus Tympanicum ; Granulation Tissue ; Humans ; Male ; Paraganglioma ; Paraganglioma, Extra-Adrenal ; Synaptophysin

This rare tumor has been diagnosed postoperatively in a 64-years-old male patient. Herein a case of extra-adrenal paraganglioma of the retroperitoneum is presented with brief review of literatures.
Humans ; Male ; Paraganglioma, Extra-Adrenal*

Paraganglioma is one of the pheochromocytomas, and this arises in the extra-adrenal tissue. It is a rare tumor of neural crest origin; it accounts for at least 10% of all the pheochromocytomas. Paragangliomas produce catecholamines and on the basis of this, they are classed as either functional or nonfunctional. We report here on our experience with laparoscopic removal of functional paragangliomas of the pelvic cavity and we include a brief review of literatures.
Catecholamines ; Laparoscopy ; Neural Crest ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Pelvis ; Pheochromocytoma

Extra-adrenal paragangliomata are uncommon entities. They can be classified into four basic groups according to their anatomical sites, i.e. branchiomeric, intravagal, aorticosympathetic and visceral autonomic. Similar tumours may arise in sites away from the usual distribution of the sympathetic and parasympathetic ganglia, e.g. orbit, nose, small intestine and even in the pancreas. We report three instructive cases of extra-adrenal paraganglioma which were found in unusual sites such as urinary bladder, thyroid gland and on the wall of the inferior vena cava.
Paraganglioma, Extra-Adrenal ; Lower case ee ; g <3> ; agreement ; inferiority

Extra-adrenal paragangliomas are rare tumors of neural crest origin, usually arising from the carotid bodies, the glomus jugulare, or the retroperitoneum. And, though ten percents of cases are presented with malignancy, it`s prognosis is relatively good. In genitourinary area, there are a few reported cases of extra-adrenal paragangliomas of the urinary bladder and the spermatic cord. Herein, we report a case of paraganglioma arising in the scrotum, which is an extremely rare location.
Carotid Body ; Glomus Jugulare ; Neural Crest ; Paraganglioma ; Paraganglioma, Extra-Adrenal* ; Prognosis ; Scrotum* ; Spermatic Cord ; Urinary Bladder

Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.
Case Report ; Corticotropin/secretion+ACo- ; Cushing Syndrome/etiology+ACo- ; Female ; Human ; Mediastinal Neoplasms/therapy ; Mediastinal Neoplasms/secretion ; Mediastinal Neoplasms/complications+ACo- ; Middle Age ; Paraganglioma, Extra-Adrenal/therapy ; Paraganglioma, Extra-Adrenal/secretion ; Paraganglioma, Extra-Adrenal/complications+ACo-

Glomuvenous malformation (GVM), an uncommon variant of venous malformation (VM), reveals dilated venous channels lined by variable amounts of glomus cells histopathologically. It appears as compressible blue or blue-purple nodules or plaque(s), sometimes showing a familial tendency. GVM usually involves only the superficial layer of the cutis or subcutaneous tissue, unlike VM, which frequently involves deeper tissue. Although GVM shares features of VM and glomus tumor, their clinicopathological and/or genetic features are sufficiently distinctive to enable a differential diagnosis, which is important for management decisions. Herein, we report five patients who showed a large confluent patch or several scattered patches composed of tender bluish blebs. Two patients among them were siblings. Histopathological and immunohistochemical examinations confirmed GVM.
Blister ; Diagnosis, Differential ; Glomus Tumor ; Humans ; Paraganglioma, Extra-Adrenal ; Siblings ; Subcutaneous Tissue

Humans ; Laparoscopy ; methods ; Male ; Middle Aged ; Paraganglioma, Extra-Adrenal ; pathology ; surgery ; Retroperitoneal Neoplasms ; pathology ; surgery

Objective: To investigate the clinicopathological features of glomuvenous malformation (GVM). Methods: Thirty-one cases of GVM diagnosed at the Henan Provincial People's Hospital from January 2011 to December 2021 were collected. Their clinical and pathological features were analyzed. The expression of relevant markers was examined using immunohistochemistry. The patients were also followed up. Results: There were 16 males and 15 females in this study, with an average age of 11 years (range, 1-52 years). The locations of the disease included 13 cases in the limbs (8 cases in the upper limbs, 5 cases in the lower limbs), 9 cases in the trunks, and 9 cases in the foot (toes or subungual area). Twenty-seven of the cases were solitary and 4 were multifocal. The lesions were characterized by blue-purple papules or plaques on the skin surface, which grew slowly. The lumps became larger and appeared to be conspicuous. Microscopically, GVM mainly involved the dermis and subcutaneous tissue, with an overall ill-defined border. There were scattered or clustered irregular dilated vein-like lumens, with thin walls and various sizes. A single or multiple layers of relatively uniform cubic/glomus cells were present at the abnormal wall, with scattered small nests of the glomus cells. The endothelial cells in the wall of abnormal lumen were flat or absent. Immunohistochemistry showed that glomus cells strongly expressed SMA, h-caldesmon, and collagen IV. Malformed vascular endothelial cells expressed CD31, CD34 and ERG. No postoperative recurrence was found in the 12 cases. Conclusions: GVM is an uncommon type of simple venous malformation in the superficial soft tissue and different from the classical glomus tumor. Morphologically, one or more layers of glomus cells grow around the dilated venous malformation-like lumen, which can be combined with common venous malformations.
Male ; Female ; Humans ; Child ; Glomus Tumor/surgery* ; Endothelial Cells/pathology* ; Paraganglioma, Extra-Adrenal/pathology* ; Immunohistochemistry