An Unusual Case of Periosteal Glomus Tumor at the Metacarpal Base Presenting as Type II CRPS: Case Report

Background: An unusual case involving a middle-aged male with a 9-year history of presumptive chronic regional pain syndrome (CRPS) and ulnar neuropathy was referred for failure in treatment. Methods: On presentation, the patient was requesting an amputation of his arm. However, work-up uncovered a periosteal extra-digital glomus tumor on the base of the small finger metacarpal. Results: Surgical excision of the lesion resulted in rapid resolution of his pain and normal hand function was ultimately restored. Conclusions: Glomus tumors account for up to 5% of all soft tissue tumors of the upper extremity, occurring most frequently within or adjacent to the nail bed. Time from onset of symptoms to correct diagnosis may not be established for many years, especially with atypical tumor locations. Although glomus tumors have been widely reported, atypical locations of these tumors should be included in the differential diagnosis for patients with unusual chronic pain or neuropathy. Furthermore, when evaluating a chronic pain patient, our findings support the opinion that assignment of the diagnosis of CRPS should only be a diagnosis of exclusion.     

Glomus tumor of the kidney: a report of 3 cases involving renal parenchyma and review of the literature

Glomus tumor is a rare mesenchymal neoplasm affecting the subcutaneous tissue of the distal extremities in the majority of cases. It only rarely involves visceral organs. We report 3 cases of the glomus tumor family in the kidney, a solid glomus tumor, a glomangioma, and a glomangiomyoma. All 3 tumors involved the renal parenchyma and occurred in 3 men aged 36, 81, and 48 years, respectively. All 3 tumors were well-circumscribed and showed morphology otherwise identical to those seen in soft tissue. All 3 tumors were immunoreactive for actin and negative for desmin and S100 and only 1 tumor expressed CD34 in tumor cells. To date, all 3 tumors have followed a benign course without evidence of recurrence or metastasis. This report expands the spectrum of mesenchymal tumors of the kidney.     

Angiogenesis in renal cell carcinoma: The role of tumor-associated macrophages

Objective:   To explore vascularity and associated molecules in renal cell carcinoma (RCC) and to study their correlations to disease outcome.
Methods:   Tissue samples from 51 Japanese patients with renal cell carcinoma (RCC) were obtained between November 1997 and August 2000. Pyrimidine nucleoside phosphorylase and vascular endothelial growth factor (VEGF) levels of RCC and normal kidney tissue were determined by enzyme-linked immunosorbent assay. Microvessel density (MVD) was measured by immunohistochemistry using anti-factor-VIII-related antigen and CD34. The number of infiltrating tumor-associated macrophages (TAM) was measured by immunohistochemistry using anti-CD68 antibody.
Results:   Pyrimidine nucleoside phosphorylase and VEGF levels were significantly higher in RCC than in normal kidney tissue. The VEGF level was higher in more progressive (high grade, larger or symptomatic) RCC. Although MVD as determined by the factor VIII level was higher in larger tumors, MVD determined by CD34 was higher in low-grade and low-stage tumors. Patients with symptoms, large tumor or high stage showed higher numbers of TAM. VEGF level and TAM were significantly higher in patients with recurrence than in those without recurrence. In univariate analysis, VEGF, TAM and CD34 tumor grade and stage were identified as prognostic factors. Moreover, TAM was the only independent prognostic factor by multivariate analysis. Among these parameters, only TAM and MVD as determined by factor VIII showed significant correlations.
Conclusion:   TAM and VEGF are substantially involved in tumor progression of RCC. As the TAM count is well correlated to the MVD, the main mechanism of tumor progression by TAM might be angiogenesis.     

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。     

Laparoscopic antral resection with Billroth I reconstruction for a gastric glomus tumor

INTRODUCTION

Gastric glomus tumors are fairly uncommon and mostly benign, with an estimated incidence of 1% of all GI soft tissue tumors. The most common GI site of involvement is the stomach, and in particular the antrum. Some cases have been discovered incidentally, but most are symptomatic presenting with GI bleeding, perforation or abdominal pain. Glomus tumors are submucosal tumors and hence mistaken with the more frequent gastrointestinal stromal tumors.

PRESENTATION OF CASE

A 33-year-old woman presented with intermittent dull upper abdominal pain for two days. Abdominal computed tomography (CT) was performed showing a hyperdense mass in the antrum. Endoscopy and endoscopic ultrasound revealed a submucosal antral mass along the greater curvature, suspicious for a gastrointestinal (GI) stromal tumor (GIST), a laparoscopic antrectomy with Billroth I reconstruction was done. Pathological examination revealed that the mass was a gastric glomus tumor.

DISCUSSION

The presented case report met all the usual standard criteria commonly used to identify glomus tumors, the uniqueness of the case lies in the occurrence of the glomus tumor in the stomach, first suspected as GIST, then confirmed as a gastric glomus tumor. The vast majority of glomus tumors of the GI tract have been described in the gastric antrum. They occur in adults of all ages with a significant female predominance (78%).

CONCLUSION

This case may aid in improving the recognition and diagnosis of this rare entity and in differentiating it from more common GISTs and gastric carcinoids. A built up knowledge between physicians is extremely necessary to avoid common confusion in taking the right medical approach.Abbreviations: CT, computed tomography; GI, gastrointestinal; GIST, gastrointestinal stromal tumor; EU, emergency unit; EUS, endoscopic ultrasound; SMA, smooth muscle actin; KIT, proto-oncogene c-Kit or tyrosine-protein kinase Kit or CD117; AFIP, Armed Forces Institute of Pathology; AUBMC, American University of Beirut Medical Center     

Subungual glomus tumors of the fingers: ultrasound diagnosis

INTRODUCTION: Glomus tumors of the fingers are rare, benign tumors. These lesions are hamartomas, originating from dermoepidermic glomus, most commonly located at the distal phalanx, and in particular under the nail. Diagnosis is often delayed because of the absence of specific symptoms. Confirmation can only be made by histological study. The aim of this retrospective study is to define the role of ultrasound examination in the diagnosis of glomus tumors of the fingers. METHOD: During the period March 2002-March 2004, 12 cases of subungueal glomus tumors were included in this retrospective study. All patients underwent ultrasound and Doppler examinations before surgery. All Ultrasound Doppler studies were performed by the same radiologist. The mean age of patients was 44 year-old and the sex-ratio was 1/2. RESULTS: Pain was noted as a symptom in all patients. The mean size of the tumor was 3.5 mm. In eleven patients, ultrasound study was able to confirm the diagnosis, but it failed in one case because of the small size of the tumor (less than 2 mm). Ten tumors presented a Doppler hypersignal, located in the tumor and compatible with the diagnosis of glomus tumor. DISCUSSION: Diagnosis of glomus tumor is based on clinical examination, standard radiology, and ultrasound Doppler study. Ultrasound examination and Doppler study gives better visualization of tumors compared to standard radiology. These studies are useful for follow-up of patients in the post-operative period.     

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.     

Multiple occurrences of different histologic types of the glomus tumor

A patient with 3 glomus tumors that occurred at different locations and times is presented. Two of the 3 tumors were of the same histologic type. The third tumor was a different type. Although it is rare, a solitary type of glomus tumor on initial presentation may exhibit multiple occurrences in the same patient.     

Upper extremity pain of 10 years duration caused by a glomus tumor

BACKGROUND AND OBJECTIVES: Long-standing limb pain typically has a multifactorial etiology not amenable to causal therapy. We present a case of chronic progressing upper extremity pain caused by a glomus tumor; the excision was curative. METHODS: A 39-year-old woman presented with 10-year history of constant deep internal throbbing, aching pain localized to the radial aspect of the left index finger and additional intermittent shooting pains radiating up the arm toward the shoulder. Pain was increased after minor local trauma, following cold exposure, and for unknown reasons. A blotchy, bluish skin discoloration could appear on the radial aspect of the index finger during severe pain. Nonsteroidal antinflammatory drugs, narcotics, amitriptyline, local heat, bracing, and a sympathetic nerve block had all been ineffective. The physical examination was characterized by exquisite pressure tenderness over the radial aspect of the left index finger, most pronounced just distal to the distal interphalengeal joint. RESULTS: Inflation of a left arm blood pressure cuff to above systolic pressures abolished pain. A glomus tumor was suspected and confirmed by histologic examination. CONCLUSIONS: Glomus tumors are rare, benign neoplasms (1 to 5% of all hand tumors), developing from neuromyoarterial glomus bodies. They usually present with pain and may mimic other painful conditions, delaying the average time until diagnosis for up to 10 years. The classic diagnostic triad consists of local pain, pressure tenderness, and cold hypersensitivity. Abolition of pain following inflation of a blood pressure cuff to above systolic levels (ischemia test) is highly diagnostic. We suggest routine use of this test in cases of upper limb pain of unclear etiology.     

32例甲外血管球瘤的临床分析

目的 总结甲外血管球瘤的临床特点及诊治方法 .方法 2000年1月至2008年4月对32例甲外血管球瘤进行诊治,其中30例单发,2例为两处病灶.均行局部病灶切除术.结果 术后随访2～98个月,平均46个月.所有患者术前症状全部消失,无复发病例.结论 甲外血管球瘤具有特征性的临床表现,即疼痛、压痛、冷激惹的三联征,因病变隐匿,诊断较困难,肿瘤切除术是唯一有效的治疗方法 .     

32例甲外血管球瘤的临床分析

目的 总结甲外血管球瘤的临床特点及诊治方法 .方法 2000年1月至2008年4月对32例甲外血管球瘤进行诊治,其中30例单发,2例为两处病灶.均行局部病灶切除术.结果 术后随访2～98个月,平均46个月.所有患者术前症状全部消失,无复发病例.结论 甲外血管球瘤具有特征性的临床表现,即疼痛、压痛、冷激惹的三联征,因病变隐匿,诊断较困难,肿瘤切除术是唯一有效的治疗方法 .     

32例甲外血管球瘤的临床分析

目的 总结甲外血管球瘤的临床特点及诊治方法 .方法 2000年1月至2008年4月对32例甲外血管球瘤进行诊治,其中30例单发,2例为两处病灶.均行局部病灶切除术.结果 术后随访2～98个月,平均46个月.所有患者术前症状全部消失,无复发病例.结论 甲外血管球瘤具有特征性的临床表现,即疼痛、压痛、冷激惹的三联征,因病变隐匿,诊断较困难,肿瘤切除术是唯一有效的治疗方法 .     

Intrapulmonary glomus tumor in a young woman

A 21-year-old female patient presented with pneumonia and on chest roentgenogram a solitary pulmonary nodule was incidentally found. After an observation period she underwent left upper lobectomy because of documented tumor growth. Pathology showed an intrapulmonary glomus tumor of the proper type, which is a very rare occurrence. Literature review revealed only 11 published cases of this subtype. Radiological investigation is helpful for localization and characterization of the tumor. However, pathological examination is required for definitive diagnosis. Complete surgical excision is the treatment of choice. Although uncommon, glomus and carcinoid tumors should be considered in the differential diagnosis of solitary pulmonary nodules in young patients.     

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.     

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     

女性手部血管球瘤的早期诊断及显微外科治疗

目的：探讨女性手部血管球瘤的临床表现,早期诊断及治疗方法。方法：选择30例女性手部血管球瘤患者,年龄18～48岁,平均35岁;病程3个月～3年。均为单指发病。早期行显微外科手术切除治疗。结果：30例中28例指甲有不同程度的局限性隆起,并可见指甲下有蓝紫色斑点状改变。18例X线片末节指骨有不同程度的受压表现。全部病例均可于指甲上找到明确并且固定的压痛点,术后患指疼痛均完全消失,经平均15个月随访,无疼痛复发病例。结论：甲下有蓝紫色斑点状改变及有固定压痛点,对女性手部血管球瘤患者有诊断意义,早期行显微外科手术切除是治疗此病有效的方法。     

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.     

The value of clinical diagnosis of digital glomus tumors

Glomus tumors are benign neoplasms that differentiate from the glomus apparatus. This neoplasm is clinically characterized by a typical symptomatological triad: paroxysmal pain, pinpoint pain and hypersensitivity to cold. The diagnosis is usually clinical, but can be confirmed by instrumental tests such as X-ray, MRI, selective arteriography and most commonly by ultrasound, with or without Doppler. Surgical resection of the glomus tumor is the definitive treatment with resolution of all symptoms. From 1997 to 2007, 21 surgically-treated cases were reviewed and here we report the clinical assessment that was used. The nine most recent cases of glomus tumor underwent surgery after clinical assessment alone. In our opinion clinical examination is the most effective method to diagnose this type of tumor and in selected cases it may be sufficient to undergo surgical therapy.     

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.     

Severe hemorrhagic syndrome in a high responder patient with hemophilia A. Value of porcine factor VIII

The case is reported of a high responder haemophiliac A, aged 37 yr, who had a severe bleeding episode after surgical treatment of a renal stone. Preoperative anti-factor VIII antibody concentration was 2 UB. Although the surgery was carried out with no real problem (partial nephrotomy), on the 5th postoperative day, there was a sudden increase in the concentration of anti-factor VIII antibodies (21 UB) with a concomitant drastic fall in Factor VIII C level, and important bleeding at the surgical site. The sequential administration of human Factor VIII, "activated prothrombin complex concentrates" (FEIBA followed by AUTOPLEX), and total nephrectomy failed to stem the haemorrhage. Despite intravenous gammaglobulins, the anti-factor VIII titre reached 1300 UB. Bleeding could only be controlled by concentrated porcine Factor VIII (HYATE-C), 100 U.kg-1.8 h-1. This treatment was continued for 50 days, with no side-effects. An average dose of 130 U.kg-1.8 h-1 maintained a residual level of Factor VIII between 1 and 23%. There was a simultaneous fall in the antibody-concentration to less than 1 UB, due to steroid treatment.