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

Objective To investigate the risk factors for patients using intraoperative vasopressor infusions during carotid body tumor(CBT)excision.Patients' mean arterial pressure(MAP)and heart rate(HR)fluctuations as well as their requirements for vasoactive agents during surgery were assessed. Methods The patients receiving CBT excision in Peking Union Medical College Hospital from May 1,2013 to July 31,2017 were included for a retrospective cohort study.The potential factors of intraoperative requirement for vasopressor infusions were investigated using univariate analysis and Logistic multivariate analysis.Furthermore,the relationships of Shamblin types of CBT with intraoperative MAP/HR fluctuations and requirements for vasoactive agents were analyzed. Results A total of 108 patients with 116 CBTs were included.Univariate analysis revealed that maximum tumor diameter >4 cm,intraoperative internal carotid artery injury,internal carotid artery reconstruction,malignant pathology,advanced Shamblin types(type Ⅱ and Ⅲ),estimated blood loss ≥400 ml,and operation duration >4 hours were associated with intraoperative requirements for vasopressor infusions.Logistic analysis showed that Shamblin type Ⅲ(OR=2.286,95% CI=1.324-14.926,P=0.016)and operation duration >4 hours(OR=3.874,95% CI=1.020-14.623,P=0.046)were risk factors for intraoperative requirements for vasopressor infusions during CBT surgery.In addition,Shamblin type Ⅲ was associated with intraoperative abnormal HR elevation and requirements for vasopressors.Conclusions Shamblin type Ⅲ and operation duration>4 hours are risk factors for intraoperative requirements of patients for using vasopressor infusions during CBT surgery.Shamblin type Ⅲ is associated with intraoperative abnormal HR elevation and requirements for vasopressors.
Carotid Body Tumor ; Humans ; Retrospective Studies ; Risk Factors ; Treatment Outcome ; Vascular Surgical Procedures

To investigate the value of head and neck CT angiography(CTA)in the evaluation of intraoperative hemorrhage of carotid body tumours. Head and neck CTA images of 36 patients with carotid body tumours confirmed by pathology were retrospectively analyzed.Patients were divided into two groups based on the intraoperative bleeding volume:<500 ml and≥500 ml groups.The patient's age,sex,Shamblin classification,size of the lesion,number of blood supply arteries,course of the disease,plain scan,and enhanced CT value between two groups were compared and analyzed.Logistics regression equation was established based on the CTA parameters with significant differences between the two intraoperative bleeding volume groups,and combined parameter was acquired.The receiver operating characteristic curve was established based on CTA single and combined parameters. The bleeding volume during the operation of carotid body tumors was significantly correlated with the age of patients(=0.019),the maximum diameter of tumours on axial images(=0.003),the maximum upper and lower diameters(=0.004),Shamblin classification(=0.012),and number of blood supply arteries(<0.001).The area under the receiver operating characteristic curve of the number of feeding arteries,the maximum diameter of axial images,maximum upper and lower diameters,Shamblin classification,and combined parameters were 0.865,0.781,0.806,0.766,and 0.927,respectively.When the optimal critical value was 0.408,the Youden index was 0.794,and the corresponding accuracy,sensitivity,and specificity were 0.919,0.909,and 0.923,respectively. Preoperative head and neck CTA can be used to evaluate the intraoperative blood loss.Combined parameters has the best diagnostic performance compared with single parameters.
Carotid Body Tumor ; diagnostic imaging ; Computed Tomography Angiography ; Head ; Humans ; Neck ; Retrospective Studies

OBJECTIVE: To analyze clinical features, surgical treatment and outcomes of neurilemmoma and carotid body tumors in bifurcation of carotid artery. METHODS: The clinical data of 17 patients with neurilemmomas and 76 patients with carotid body tumors at the bifurcation of carotid artery, who were surgically treated in Zhongshan Hospital of Fudan University from March 2012 to November 2016, were retrospectively analyzed. The clinicopathological characteristics, surgical procedures and outcomes were compared between two groups. RESULTS: No difference of preoperative clinical demographic data was found between two groups. Operation time of the neurilemmoma group was significantly shorter than that of the carotid body tumor group[(93.9±30.8) min vs. (159.3±52.9) min, <0.01]. The neurilemmoma group had lower volume of intra-operative blood loss[(110±96) mL vs. (356±239) mL, <0.01] and lower rate of external carotid artery resection (11.8% vs. 68.4%, <0.01) than the carotid body tumor group. In the neurilemmoma group, 17 tumors were completely resected and no malignant disease was found. In the carotid body tumor group, 76 patients underwent complete surgical resection for the tumor, of which 5 (6.6%) were malignant. Tumor size of the neurilemmoma group was larger than that of the carotid body tumor group[(4.5±1.4) cm vs. (3.1±1.0) cm, <0.01]. There was no significant difference in the incidence of peri-operative complications and length of hospital stay between two groups (>0.05). CONCLUSIONS The clinical manifestations of neurilemmoma and carotid body tumors at carotid artery bifurcation are similar. The carotid body tumor group has a longer operating time, larger intra-operative blood loss, higher external carotid resection rate and relative higher incidence of malignancy. More cautions should be given when carotid body tumors at carotid artery bifurcation are treated.
Carotid Arteries ; surgery ; Carotid Body Tumor ; pathology ; surgery ; Humans ; Neurilemmoma ; pathology ; surgery ; Retrospective Studies ; Treatment Outcome

Paraganglioma in pregnancy is an extremely rare condition and its diagnosis is often delayed because the clinical symptoms can mimic those of preeclampsia or gestational hypertension. Here, we report the case of a 32-year-old, gravida 2, para 1 woman who presented with severe headache, palpitation, and sweating at 37 weeks' gestation. Although emergent cesarean section was performed on the assumption of severe preeclampsia, blood pressure fluctuated and heart rate remained tachycardiac. We suspected that she might have thromboembolic lesion in the chest or pheochromocytoma. Chest and abdominal computed tomography revealed a 4 cm mass in the left para-aortic space. Serum and urinary catecholamine levels were found to be significantly increased. She underwent laparoscopic mass removal and the pathology confirmed paraganglioma. When typical paroxysmal hypertension is accompanied by headache, palpitation, and sweating during pregnancy, adrenal tumors should be considered.
Adult ; Blood Pressure ; Cesarean Section ; Diagnosis ; Female ; Headache ; Heart Rate ; Humans ; Hypertension ; Hypertension, Pregnancy-Induced ; Paraganglioma ; Paraganglioma, Extra-Adrenal* ; Pathology ; Pheochromocytoma ; Pre-Eclampsia* ; Pregnancy ; Sweat ; Sweating ; Thorax

Carotid Artery, Internal ; physiology ; Carotid Body Tumor ; blood supply ; complications ; surgery ; Carotid Sinus ; physiopathology ; Female ; Humans ; Middle Aged ; Syncope ; etiology ; Syndrome

Primary meningioma of jugular foramen is extremely rare, while paraganglioma or nerve sheath tumor are relatively common in jugular foramen. We reported a case of primary meningioma of jugular foramen. A 79-year-old female who had left tinnitus and hearing loss for three month came to the department of otorhinolaryngology. Temporal bone computed tomography scan showed sclerotic change and slightly irregular margins of left jugular foramen with relatively preservation of bony architecture. Temporal bone magnetic resonance image showed well defined homogeneous enhancing mass in left jugular foramen with extension to carotid space on gadolinium enhanced T1 weighted image. Prominent dural tail was also noted. On T2 weighted image, this mass showed intermediated signal intensity with no vascular signal voids. Meningioma was confirmed by pathology. In this article, we describe a case of primary en plaque meningioma of jugular foramen and review image findings of differential diagnosis.
Aged ; Diagnosis, Differential* ; Female ; Gadolinium ; Glomus Jugulare Tumor ; Hearing Loss ; Humans ; Meningioma* ; Neurilemmoma ; Otolaryngology ; Paraganglioma ; Pathology ; Tail ; Temporal Bone ; Tinnitus

OBJECTIVES: To describe a rare case of chemodectoma, its clinical features and management, and to discuss its relationship chronic hypoxia from Tetralogy of Fallot.

METHODS:
Design: Case Report

Setting: Tertiary Government Hospital

Patient: One

RESULTS: A 23-year-old woman presented with a painless, slow growing, movable right submandibular mass, initially diagnosed as a lipoma by fine needle aspiration biopsy.  Computed tomography scan showed a solid nodule with ill-defined margins from the angle of the mandible to the level of the hyoid bone along the carotid sheath. There was also an incidental finding of patent ductus arteriosus and Tetralogy of Fallot on pre-operative clearance. Excision of the mass under general anesthesia revealed adherence to the posterior portion of the carotid trunk enveloping both the internal & external carotid artery. Final histopathological diagnosis was chemodectoma.

CONCLUSION: Although rare, chemodectoma should be considered as one of the differentials in a patient with a submandibular mass. Hyperplastic chemodectoma may result from chronic hypoxia due to Tetralogy of Fallot.  Surgical excision is the treatment of choice.

Human ; Female ; Adult ; Carotid Body Tumor ; Paraganglioma ; Tetralogy of Fallot

OBJECTIVES: To present a case of type 1 glomus tympanicum, its clinical presentations, surgical management and outcome.
METHODS:
Design: Case Report
Setting: Tertiary Government Hospital
Patient: One
RESULTS: A 44-year-old woman with pulsatile tinnitus, vertigo, headache, ear fullness and decreased hearing on the right had a pulsatile reddish mass behind the tympanic membrane and Brown sign. Weber test lateralized to the right with mild conductive hearing loss on pure tone audiometry. Contrast CT scan demonstrated a 5x6 mm well-defined enhancing mass in the meso- and hypotympanum. Internal auditory canal MRI showed an avidly enhancing 5x3x4 mm nodule within the right middle ear adjacent to the cochlear promontory and anterior to the lateral semicircular canal. Impression was glomus tympanicum, type 1. The mass was excised via transcanal approach with post-operative resolution of tinnitus, headache, vertigo and improvement of hearing. Final histopathology was consistent with glomus tumor.
CONCLUSION: Glomus tympanicum tumors are rare, benign middle ear paragangliomas that arise from Jacobson's nerve are slow-growing and locally destructive. CT scan and MRI may detect involvement of other structures. Surgical resection is the primary treatment modality. Type 1 glomus tympanicum tumors are small and limited to the promontory and a less-invasive transcanal approach may be employed.

Human ; Female ; Adult ; Glomus Tympanicum Tumor ; Tympanic Membrane ; Hearing Loss, Conductive ; Glomus Tumor ; Ear, Middle ; Hearing Loss ; Ear Neoplasms ; Vertigo

Paraganglioma is an uncommon and slowly-growing benign neuro-endocrine tumor originating from the extra-adrenal paraganglia. The most common site of head and neck paraganglioma is the carotid body. Paraganglioma of thyroid gland is a rare disease, and is hard to diagnose preoperatively since the pathologic features are similar to thyroid neoplasm, especially to medullary carcinoma. Occasionally, multiple paragangliomas can be found in the body. To our knowledge, there are two cases of synchronous carotid body tumor with thyroid paraganglioma that have been reported to date. We report a case of 34-year-old female with carotid body paraganglioma synchronous with intra-thyroid paragangloma, which was misdiagnosed as papillary thyroid carcinoma in the preoperative evaluation. After the tumors were successfully excised, there were no signs of complications and the patient has survived the 2-year-follow-up period without recurrence.
Adult ; Carcinoma, Medullary ; Carotid Body ; Carotid Body Tumor* ; Female ; Head ; Humans ; Neck ; Paraganglioma* ; Rare Diseases ; Recurrence ; Thyroid Gland ; Thyroid Neoplasms