Long-term results of surgery for temporal bone paraganglioma

The only way to resolve the dispute about the effectiveness of surgery versus radiation therapy for glomus tympanicum and jugulare tumors is adequate long-term studies. In a retrospective study with an average follow-up period of 15 years (range 11 to 23 years) we reassessed 11 patients with glomus tympanicum tumors and 11 patients with glomus jugulare tumors. Ten of 11 patients with glomus tympanicum tumor were tumor-free after surgery. A temporary facial palsy and an external meatal wall defect were the only surgical complications. The air-bone gap postoperatively closed to within 10 dB in three patients, to within 20 dB in six patients, and to more than 30 dB in one patient. Nine of 10 patients with glomus jugulare tumor receiving complete resection were tumor-free. Less than half the patients experienced new-onset cranial nerve function loss, and all made satisfactory recovery, eliminating the need for tracheostomy or gastrostomy. In two patients, the hearing could be preserved on the preoperative level, but the majority already presented with deafness. In the long-term, surgery remains a treatment of choice for glomus tympanicum tumors. It is also an extremely effective treatment with low morbidity for glomus jugulare tumors, including those with intracranial extension.     

Long-Term Results of Surgery for Temporal Bone Paraganglioma

The only way to resolve the dispute about the effectiveness of surgery versus radiation therapy for glomus tympanicum and jugulare tumors is adequate long-term studies. In a retrospective study with an average follow-up period of 15 years (range 11 to 23 years) we reassessed 11 patients with glomus tympanicum tumors and 11 patients with glomus jugulare tumors. Ten of 11 patients with glomus tympanicum tumor were tumor-free after surgery. A temporary facial palsy and an external meatal wall defect were the only surgical complications. The air-bone gap postoperatively closed to within 10 dB in three patients, to within 20 dB in six patients, and to more than 30 dB in one patient. Nine of 10 patients with glomus jugulare tumor receiving complete resection were tumor-free. Less than half the patients experienced new-onset cranial nerve function loss, and all made satisfactory recovery, eliminating the need for tracheostomy or gastrostomy. In two patients, the hearing could be preserved on the preoperative level, but the majority already presented with deafness. In the long-term, surgery remains a treatment of choice for glomus tympanicum tumors. It is also an extremely effective treatment with low morbidity for glomus jugulare tumors, including those with intracranial extension.     

Glomus tumor originating within a digital nerve

Glomus tumors are thought to arise from specialized cells normally found within the glomus apparatus of the dermal stratum reticularis. We report the unusual occurrence of a glomus tumor from within a peripheral nerve. Previous investigators have suggested that the rare occurrence of glomus tumors in tissue where the glomus apparatus has not been described arise from ectopic glomus cells or differentiate from nonglomus cell sources. Many of the "ectopic" glomus tumors have been found in regions of extensive arteriovenous shunting such as in the digestive system and lung suggesting the presence of glomera at these locations. The finding of a glomus tumor within a peripheral nerve, a region where extensive arteriovenous shunting and glomus bodies have not been described, supports the occasional origin of a glomus tumor from a nonglomus cell source.     

Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors

Occasional glomus tumors display unusual features, such as large size, deep location, infiltrative growth, mitotic activity, nuclear pleomorphism, and necrosis. Although a small number of purportedly malignant glomus tumors have been described, histologic criteria for malignancy in glomus tumors have never been elaborated. The authors studied 52 unusual glomus tumors (retrieved from their consultation files) previously diagnosed as "atypical" or "malignant" by virtue of nuclear atypia, infiltrative growth, or mitotic activity. They evaluated size, depth, growth pattern, cellularity, nuclear grade, number of mitotic figures per 50 high-power fields (HPF), atypical mitotic figures, vascular space involvement, and necrosis to define criteria for malignancy in glomus tumors. Estimated relative risk was calculated and the Fisher exact test was used for statistical analysis. The 27 female patients and the 25 male patients ranged in age from 8 to 83 years (median age, 43 years). The tumors measured from 0.2 to 12 cm (median size, 2 cm) and occurred predominantly in the extremities, in both the superficial (n = 35) and deep (n = 17) soft tissues. Atypical features were usually observed centrally with a rim of benign-appearing glomus tumor. Follow-up information (n = 35; range, 5 months-23 years; mean 5.5 years) showed seven recurrences, eight metastases, and seven deaths from disease. Five-year cumulative metastatic risk increased significantly for tumors with a deep location (p = 0.005), with a size of more than 2 cm (p = 0.004), and with atypical mitotic figures (p = 0.004). Mitotic activity of more than 5 mitoses/50 HPF, high cellularity, the presence of necrosis, and moderate to high nuclear grade approached but did not reach significance. High nuclear grade alone, infiltrative growth, and vascular space involvement were not associated with metastasis. The authors propose the following classification scheme and criteria. Malignant glomus tumor: Tumors with a deep location and a size of more than 2 cm, or atypical mitotic figures, or moderate to high nuclear grade and > or =5 mitotic figures/50 HPF. Symplastic glomus tumor: Tumors with high nuclear grade in the absence of any other malignant feature. Glomus tumor of uncertain malignant potential: Tumors that lack criteria for malignant glomus tumor or symplastic glomus tumor but have high mitotic activity and superficial location only, or large size only, or deep location only. Glomangiomatosis: Tumors with histologic features of diffuse angiomatosis and excess glomus cells. Using this classification scheme, metastasis was observed in 38% of tumors fulfilling the criteria for malignancy. In contrast, metastatic disease was not seen in any specimen classified as symplastic glomus tumor, glomus tumor of uncertain malignant potential, or glomangiomatosis.     

Primary fallopian canal glomus tumors

Primary fallopian canal glomus tumor has been reported only once previously, although the occurrence of glomus body tissue in the fallopian canal was documented many years ago. Facial paresis as a presenting symptom of glomus tumors is well known, as is facial nerve invasion by glomus tumors. However, a primary fallopian canal glomus tumor that extends extratemporally to the pes anserinus is unusual. Although facial nerve grafting may be necessary for removal of some glomus jugulare tumors, the need for facial nerve grafting appears to be uniform in the patients with primary fallopian canal glomus tumors. The primary fallopian canal glomus tumors that we report did not involve the jugular fossa or the Jacobson's branch of the glossopharyngeal nerve. Both tumors did extend to the middle ear and mastoid and followed the facial nerve extratemporally. The latter features appear to typify primary fallopian canal glomus tumors.     

Glomangiomyoma of the trachea

A glomus tumor is an uncommon soft tissue tumor that is most commonly found in the subungual area and a glomus originating in the trachea is extremely rare. Histologically and ultrastructurally, these tumors have been divided into three subtypes: classic glomus tumors, glomangiomas, and glomangiomyomas. Glomangiomyomas account for less than 10% of all glomus tumors and are the least common type. We report a case of a 54-year-old man with glomangiomyoma of the trachea who presented with stridor. We treated the tumor by segmental resection and primary repair via a transcervical approach.     

Paraganglioma of the cranial vault. Apropos of a case]

The authors report the case of a 40 years old woman with a fronto parietal tumefaction and intracranial hypertension caused by a paraganglioma of the cranial vault. The clinical follow up is marked by rapid local recurrence of the tumor after complete excision. Usually paragangliomas arise in any area of the body where paraganglionar structures are situated (glomus jugulare, carotid glomus, vagus nerve). Other rare locations have been reported and remain difficult to be explained. The location at the cranial vault is exceptional. this tumor would probably arise from the paraganglionar cells which migrate from the neural crest following the trigeminal branches particularly the ophtalmic division.     

手指血管球瘤26例临床分析

目的　探讨手指血管球瘤的诊断和手术方法。方法　 1996年 1月 -2 0 0 2年 12月 ,对 2 6例手指血管球瘤进行诊治。术前 2 6例Love氏试验阳性 ,冷敏感试验阳性 ,2 0例Hildreth氏试验阳性 ,15例中 11例透光试验阳性 ,1例X线侧位片显示末节指骨有肿瘤压迹。全部病例均采取肿瘤切除手术。结果　术后随访 11个月～ 7年 9个月 ,无 1例肿瘤复发。 2 6例疼痛完全消失 ,术前阳性症状全部消失。 2 2例指甲生长良好 ,无明显畸形。结论　提高对血管球瘤的认识 ,有助于早期诊断 ,早期治疗 ,完整切除肿瘤是提高临床疗效的关键。     

Multiple glomus tumors of the stomach with intravascular spread.

Gastric glomus tumors are characteristically benign solitary lesions. We present a case of multiple glomus tumors involving the stomach wall and perigastric adipose tissue. Histologically, the major portion of each tumor was composed of "typical" glomus cells arranged in a predominantly solid pattern. Cavernous hemangiomalike areas were present toward the periphery. The tumor cells were immunoreactive for alpha-smooth muscle actin, vimentin, laminin, and type IV collagen, but did not express desmin. There were several focal areas where the tumor cells had a signet-ring cell-like appearance, intermingled with cells having clear cytoplasm and hyperchromatic nuclei. Rare mitoses were noted. A striking feature was the presence of widespread but subtle extension of the glomocytes along venous channels subendothelially, with formation of intravascular nodules focally. The multiple separate tumor nodules found in perigastric fat are interpreted as having arisen in this manner. This entity is distinct from previously described typical and atypical solitary glomus tumors, glomangiosarcoma, and the syndrome of multiple glomus tumors of subcutaneous tissues. It may represent an early stage of development of the large, multilobulated glomus tumors that have rarely been reported to involve stomach and adjacent viscera.     

Glomus tumors

The authors report a series of 12 consecutive patients with 13 glomus tumors operated from July 1991 until February 1999. Symptoms were present for an average of 1.9 years before surgery. Women were more frequently affected. The mean age was 44 years. In 12 of the 13, the tumor was located in the distal phalanx and one patient had a glomus tumor on the dorsum of the hand. One glomus tumor was found in the right hallux of a two-year-old child. Both hands and all fingers were equally involved. One bilateral glomus tumor was associated with neurofibromatosis. All tumors were resected and histology confirmed the diagnosis. The result was good with immediate pain relief. No recurrence has been noted to date.     

Definitive radiotherapy in the management of chemodectomas arising in the temporal bone, carotid body, and glomus vagale

PURPOSE: To evaluate the results of treatment for 71 patients with 80 chemodectomas of the temporal bone, carotid body, or glomus vagale who were treated with radiation therapy (RT) alone (72 tumors in 71 patients) or subtotal resection and RT (8 tumors) at the University of Florida between 1968 and 1998. METHODS AND MATERIALS: Sixty-six lesions were previously untreated, whereas 14 had undergone prior treatment (surgery, 11 lesions; RT, 1 lesion; or both, 2 lesions) and were treated for locally recurrent disease. All three patients who received prior RT had been treated at other institutions. Patients had minimum follow-up times as follows: 2 years, 66 patients (93%); 5 years, 53 patients (75%); 10 years, 37 patients (52%); 15 years, 29 patients (41%); 20 years, 18 patients (25%); 25 years, 12 patients (17%); and 30 years, 4 patients (6%). RESULTS: There were five local recurrences at 2.6 years, 4.6 years, 5.3 years, 8.3 years, and 18.8 years, respectively. Four were in glomus jugulare tumors and one was a carotid body tumor. Two of the four patients with glomus jugulare failures were salvaged, one with stereotactic radiosurgery and one with surgery and postoperative RT at another institution. Two of the five recurrences had been treated previously at other institutions with RT and/or surgery. Treatment for a third recurrence was discontinued, against medical advice, before receiving the prescribed dose. There were, therefore, only 2 failures in 65 previously untreated lesions receiving the prescribed course of RT. The overall crude local control rate for all 80 lesions was 94%, with an ultimate local control rate of 96% after salvage treatment. The incidence of treatment-related complications was low. CONCLUSIONS: Irradiation offers a high probability of tumor control with relatively minimal risks for patients with chemodectomas of the temporal bone and neck. There were no severe treatment complications.     

Gastrointestinal glomus tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases

Glomus tumors usually occur in the peripheral soft tissues, but similar tumors have also been reported in the stomach and occasionally in the intestines. However, the relationship of these tumors to peripheral glomus tumors and gastrointestinal stromal tumors has not been fully clarified because previous series of gastrointestinal glomus tumors predate availability of immunohistochemistry. This clinicopathologic study examined 32 gastrointestinal glomus tumors. All but one of the tumors were located in the stomach and the remaining tumor was from the cecum. The tumors occurred with a strong female predominance (23 females and 9 males) and a median age of 55 years (range 19-90 years). The gastric tumors typically presented with gastrointestinal bleeding or ulcer-like symptoms, and 14 tumors had mucosal ulceration. Five tumors were incidental findings. The tumor sizes varied from 1.1 to 7 cm (median 2 cm), and most were located in the antrum. Histologically, the tumors typically had a solid pattern of sharply demarcated, round glomus cells with prominent, mildly dilated pericytoma-like vessels. Vascular invasion and focal atypia were relatively common (seen in 11 and 13 cases, respectively), and low mitotic activity (1-4 per 50 high power fields), was seen in 10 cases. Immunohistochemically, all tumors were positive for alpha-smooth muscle actin and calponin, and nearly all had a net-like pericellular laminin and collagen type IV positivity. All tumors were negative for desmin and S-100 protein. Three tumors had focal synaptophysin positivity, but none was positive for chromogranin. All tumors lacked KIT expression and the GIST-specific mutations in the c-kit gene. Follow-up revealed one patient death of metastatic disease to liver at 50 months; this tumor had 1 mitosis per 50 high power fields, but had spindle cell foci, mild atypia, and vascular invasion. Thirteen patients were well and alive after long-term follow-up. Gastrointestinal glomus tumors occur almost exclusively in the stomach, and they have a good overall prognosis, but a small, unpredictable potential for malignant behavior exists. These tumors are phenotypically similar to peripheral glomus tumors and differ from epithelioid GISTs.     

Glomangiosarcoma of the hypothenar eminence: a case report

Glomangiosarcoma is an exceptionally rare soft tissue tumor. It tends to appear as a painful nodule located in the subcutaneous tissue. There are only two cases described on the hand before instead of the benign glomus tumor is usually located at this level. Histochemically the glomangiosarcoma shows features that remind a benign glomus tumor, except for the malignant glomus tumor arising de novo. This neoplasm is considered a low grade malignant tumor with tendency to local recurrence, though metastasis have been reported. We report the case of a 36 year-old -woman with a glomangiosarcoma in a glomus tumor in the hypotenar eminence. The treatment was a local complete excision and there was no signs of recurrence after 18 months.     

手部血管球瘤的诊断与显微切除术

目的 探讨手部血管球瘤诊治的临床经验。方法 1994年以来，我院共收治手部血管球瘤患者72例，确诊后全部行显微手术切除，术后病理证实。结果 术后效果良好，随访3个月～10年，无1例复发。结论 诊治的成功关键在于：血管球瘤的临床三联症表现，手术采用显微技术完整切除瘤体，是防止复发的关键。     

Popliteal glomangioma mimicking baker’s cyst in a 9-year-old child: an unusual location of a glomus tumor

Popliteal cysts (Baker’s cyst) are very common conditions in children. However, in the differential diagnosis of popliteal cysts in childhood, glomus tumor is the most infrequent diagnosis. This is a relatively uncommon soft tissue tumor that can occur at any age and any anatomic site, with a predilection for the subungual region. In the reported series, extradigital glomangiomas involving the knee are located in the patellar ligament, quadriceps tendon, patellar fat pad, and popliteal fossa. In this study, I present a second case of glomus tumor located in the popliteal fossa and misdiagnosed as a Baker’s cyst. I also review the “around-the-knee glomus tumors” that have been discussed in the literature to date.     

Pediatric glomus tumors

Glomus tumors of the middle ear are unusual in adults, but exceedingly rare in children. While a dull, red bulging tympanic membrane in the adult may suggest a glomus tumor, it generally signifies infection in the child. This report details our management of a 10-year-old girl afflicted with bilateral chronic middle ear cleft infection that obscured bilateral glomus tumors. Review of the current English language literature reveals seven additional case reports of otologic glomus tumors in children less than fourteen years of age. Two additional cases are presented that were given to the senior author by personal communication, producing a total of 10 cases for review. Glomus tumors in children may be hidden by otitis media and appear more likely to be endocrine active. Failure to cure the lesion is apparent in five of six case reports of children with glomus jugulare tumors; three of these children are reported to have expired. Complete surgical extirpation is advocated for childhood glomus tumors.     

Efficiency of gamma knife radiosurgery in the treatment of glomus jugulare tumors.

This study was performed to assess the impact of gamma knife radiosurgery (RS) in the treatment of glomus jugulare tumors. Between February 1993 and February 1999, thirteen patients (9 women, 4 men; mean age 63.5 years, range 29 to 79 years) underwent stereotactic radiosurgery for glomus jugulare tumors with the Leksell Gamma Knife at the Neurosurgical Department of the University of Vienna. Four patients, mean age 74.5 years, range 67 to 79 years, underwent radiosurgery as the only treatment. Nine patients received radiosurgery as adjuvant therapy after previous treatment had failed: surgical resection in 9 patients and additional fractionated external beam radiation in two of these patients. Pretreatment evaluation included the staging of all tumors according to the Fisch Classification: De1 (7), De2 (1), Di1 (4) and Di2 (1). The mean follow-up period was 4.2 years, range 0.7 to 6.7 years. Ten patients, 77 %, were treated prior to 1997, the mean follow-up period being 5 years. Six patients showed no clinical changes, while six experienced an improvement of their clinical symptoms and neurological deficits. One patient was lost to follow-up. Radiation-induced transient cranial nerve neuropathies were observed in two patients. Serial MRI scans revealed tumor control in all patients, with unaltered tumor size in 10 and shrinkage in three patients. The results indicate that RS is an attractive treatment option for glomus jugulare tumors and will occupy an increasingly important role in the management of these tumors in selected patients.     

Glomus Tumor in a Segmental Bronchus: A Case Report

Glomus tumors originate from a neuroarterial structure called the glomus body, and grow mostly in soft tissue. It is rare for glomus tumors to develop in the respiratory system. The patient of the present case had an abnormal shadow in the right lung on chest X-ray, and computed tomography (CT) findings displayed a lung tumor in the right S6. Bronchoscopy was performed for the diagnosis of the lung tumor, and a polypoid bronchial tumor was unexpectedly found to occupy the right B3. The bronchial tumor was diagnosed as a glomus tumor, and the lung tumor was diagnosed as an adenocarcinoma. The bronchial glomus tumor was cauterized by argon plasma coagulation (APC). Three weeks after the cauterization by APC, the right lower lobectomy was performed for the treatment of the lung adenocarcinoma. The patient has remained disease free for 2 years.     

Familial glomus tumor encountered in the same finger and localization in four family members

A 35-year-old man was referred to our clinic by a neurosurgeon for hypersensitivity and severe pain in his right middle finger. History showed that the symptoms had been present for three years and he had made several attempts to seek medical attention. A sensitive, very painful mass was palpated under the eponychium of his right middle finger. The tumor was surgically removed and histological examination confirmed the clinical diagnosis of a glomus tumor. Inquiry into the family history revealed that the same problem existed in the same localization with similar complaints in the father and two daughters. Both the records of the father's operation 10 years before and clinical-radiological findings of the daughters were consistent with a familial entity. In the presence of hypersensitivity and pain in the finger, family members should be inquired and a diagnosis of familial glomus tumor should be considered.