Glomus tumors of the hand

Summary Experience with 15 cases of glomus tumors of the hand is reported. This condition is described as being uncommon but is not so rare as is commonly stated. Many are misdiagnosed or overlooked because of unfamilarity with the condition.This paper was presented in The Fifth Congress of the Asian Pacific Section, in September 1989, Istanbul, Turkey     

Solitary Epicranial Neurofibroma with Neurofibromatosis Type 1-Related Germline Mutation: Case Report

A 33-year-old male became aware of a painless soft mass in the left occipital region. His medical and family history were unremarkable for neurofibromatosis type 1 (NF1) or other genetic disorders. Physical examination showed no signs of NF1. Neurological and ophthalmological examinations found no abnormality. Cranial computed tomography showed an isodense mass located subcutaneously with irregular deformities in the adjacent occipital bone. Magnetic resonance (MR) imaging demonstrated that the lesion, 7.5 × 5.5 cm in diameter, was hypointense both on T₁- and T₂-weighted images and intensely enhanced after gadolinium infusion. The patient requested to remove the large mass. The subcutaneous tumor was well circumscribed, encapsulated, and less vascular, and resected en bloc. The histological diagnosis was neurofibroma without findings of cell atypia, whereas genomic exploration identified abnormal gains in NF1 gene, and resultant absence of neurofibromin, a protein coded on NF1 gene. Solitary neurofibromas in “clinically” non-NF1 patients may originate from the genomic changes in NF1 gene.     

Treatment of Recurrent and Residual Glomus Jugulare Tumors

Residual and recurrent glomus jugulare tumors are rare but challenging. Treatment options include microsurgical resection, stereotactic radiotherapy, a combination of modalities, and “observation.” Choice of treatment must be made on a case-by-case basis, considering patient age, health status, location and size of tumor, status of the lower cranial nerves, and, of course, patient desire. Surgery is preferred when total resection of the tumor with preservation of function is deemed achievable. When function of the lower cranial nerves has been compromised, total surgical resection may also be possible, provided that the patient's health allows it. Cases where function is still preserved despite presence of a large tumor are more challenging, and a combination modality may be most effective. The goal of treatment is to provide tumor control with low morbidity. Current surgical techniques and the availability of stereotactic radiotherapy make this possible in the majority of cases.     

Glomus tumor of the stomach

A case of glomus tumor of the stomach, found 7 cm oral to the pylorus of a 23-year-old man was reported.     

Glomus tumors

Glomus tumors are rare vascular tumors that are found most commonly in the hand. Although the tumors are benign, they can cause intense focal pain. Patients often go undiagnosed or misdiagnosed for many years because the tumors are small, usually not palpable, and have varying presentations. A combination of magnetic resonance imaging and clinical examination is used to support the diagnosis. Surgical excision normally provides complete pain relief. An awareness of the diagnosis is critical to preventing an unnecessary delay in treatment.     

Glomus tumor of the mesocolon

Glomus tumors are rare distinctive benign neoplasms, which arise from modified smooth muscle cells of the normal glomus body and are most commonly located in the subungual region of the finger. Intraabdominal locations are relatively rare. We report a case of glomus tumor of the mesocolon in a 10-year-old girl. Surgical exploration showed a lesion in the transverse mesocolon, which was excised. Histopathology showed it to be a glomus tumor of the mesocolon.     

Superficial Fibromatosis Mimicking Glomus Tumor of the Second Toe

Various types of tumor can occur in the subungual space, including glomus tumors, subungual exostosis, hemangioma, epidermal cysts, and malignant tumors. While fibromatosis can occur at various sites throughout the body, it is very rarely seen in the toe. Here, we are the first to report a case of superficial fibromatosis mimicking a glomus tumor in the subungual space of the second toe. The presentation of this condition shows the possibility of encountering uncommon superficial fibromatosis in the distal phalanx of the toe, and suggests that superficial fibromatosis should be included in the differential diagnosis of a glomus tumor in the toe.     

Glomus tumor of the lateral aspect of the knee joint

Background We report a rare case of glomus tumor that occurred in the lateral joint of the knee. A 54yearold man was referred to us with a 3year history of lateral pain in the left knee and the diagnosis of lateral meniscus injury. Physical examination revealed a small trigger point localized just on the lateral joint space. Magnetic resonance imaging (MRI) showed a very small round mass in the subcutaneous tissue at the level of the lateral joint space.Treatment Excisional biopsy was performed, and the histology was characteristic of a glomus tumor. The patient was asymptomatic immediately after the operation. At the 3-year followup after the operation, no recurrence was seen.No benefits in any form have been received or will be received from any commercial party related directly of indirectly to the subject of this article. No funds were received in support of this study.     

The Incidence of Scoliosis Due to Neurofibromatosis

Cobb in 1950 stated that a high proportion of scolioses, classified as idiopathic, may be due to neurofibromatosis. This paper describes a retrospective study of a series of 3209 scoliosis cases to determine the incidence of neurofibromatosis. It was found to be just over 3 per cent. Two different clinical pictures of scoliosis were seen among the neurofibromatosis cases.     

Synaptophysin Positive Glomus Tumor of Trachea Simulating Typical Carcinoid: A Potential trap

Glomus tumors (GTs) are uncommon benign tumors, accounting for <2% of all soft tissue tumors and usually occur within the dermis or subcutis of distal extremities. Primary tracheal GT is rare, and to date less than 40 tracheal GTs have been reported. Tracheal GT usually present as a polypoidal mass. They express smooth muscle markers, and are negative for cytokeratin (CK) and neuroendocrine markers on immunohistochemistry (IHC). We here report a case of tracheal GT showing diffuse strong aberrant immunoexpression for synaptophysin, initially construed as carcinoid. Focal synaptophysin expression has been described in few gastric GTs, and a nasal GT. Diligent histomorphological examination and careful selection of IHC panel helps in clinching the diagnosis. Complete resection is the standard treatment of modality.     

Successful Resection of locally infiltrative Glomus Tumor without pulmonary resection

IntroductionExtracutaneous glomus tumors occurring in the bronchus is very rare. Complete resection is basic procedure for treatment of glomus tumor. We present a glomus tumor of the left main bronchus that was successfully treated with rigid bronchoscopy followed by sleeve resection of the left main bronchus.Presentation of caseA 56-year-old man underwent two term resections to glomus tumor that originated from the left main bronchus. Firstly, we performed palliative resection with rigid bronchoscopy to make the correct diagnosis and evaluate the extent of the tumor. We subsequently performed curative resection. No complications or recurrence has occurred since the operation took place one year ago.DiscussionBefore curative resection, it is important to confirm the diagnosis and spread of the tumor. Therefore, palliative tumor resection by rigid bronchoscopy was useful to make the correct diagnosis, evaluate the extent of the tumor and open the bronchial lumen. After bronchoscopic treatment, curative pulmonary resection was performed and preservation of lung function was successful.ConclusionTwo term resections enabled us to make an accurate diagnosis and evaluation, thereby preserving respiratory function without pulmonary resection.     

Glomus Tumor of Hoffa's Fat Pad and Its Management by Arthroscopic Excision

We present a rare case of glomus tumor of Hoffa''s fat pad in a 42-year-old woman. Magnetic resonance imaging findings along with the characteristic clinical picture led us to suspect a glomus tumor as the possible etiology. An ischemia test was found to be positive and this further substantiated our diagnosis. An arthroscopic excision was performed and the histology confirmed the diagnosis of glomus tumor of Hoffa''s fat pad. The patient responded well to the excision with immediate complete resolution of pain and she remains asymptomatic at the last follow-up after 15 months. To our knowledge, this is the second reported case of glomus tumor of Hoffa''s fat pad and the first ever to be managed by simple arthroscopic excision. The tumor poses a great challenge to an orthopedic surgeon. However, knowledge of its characteristic clinical presentation and the recognition of such a rare entity can help achieve an early diagnosis and timely management.     

Glomus tumors of the hand in the Netherlands: analysis of 107 patients

The aim of this article is to analyze the presenting symptoms, localization, treatment and number of recurrences of glomus tumors of the hand in the Netherlands. The data of patients with a glomus tumor of the hand, diagnosed between January 1994 and February 1998 were provided by PALGA, a pathology foundation which registers all pathology diagnoses in the Netherlands. The patients’ names were not disclosed. Each physician who operated on these patients received a questionnaire. In the period from 1994–1998, 107 patients were diagnosed with a glomus tumor of the hand. Significantly more females than males were affected. In half of the cases, the tumors were located subungually. In only 56% of the cases did the operating physician included ”glomus tumor” in the differential diagnosis. The mean period between start of the symptoms and excision of the lesion was approximately 4 years. The follow-up duration ranged from 4 weeks to 5 years. After excision, all patients but one were symptom free. In all, there were five recurrences (5%). The most significant finding in this review was a considerable delay in diagnosis and treatment of this tumor. Received: 9 June 1999 / Accepted: 9 August 1999     

The Spectrum of Neurofibromatosis: Neurologic Manifestations with Malignant Transformation

ABSTRACT

Neurofibromatosis is an uncommon cause of tetraplegia. Spinal cord compression in association with brachial plexus involvement is rare. A case of neurofibromatosis with malignant transformation illustrates the neurologic features associated with this condition. Malignant transformation of a neurofibroma should always be considered in patients with recurrent lesions or progressive neurologic dysfunction.     

Segmental neurofibromatosis of the hand and upper extremity: a case report

A case of segmental neurofibromatosis of the upper extremity is presented. Multiple neurofibromas involving different peripheral nerves limited to a single body part or limb is a rare form of neurofibromatosis. The clinical, genetic, and histologic findings of segmental neurofibromatosis are described. The criteria for segmental neurofibromatosis are reviewed, and the differentiation of neurofibromatosis types 1 and 2 and schwannomatosis is also briefly reviewed.