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Supraclavicular glomus tumor, 20 year history of undiagnosed shoulder pain: a case report
Pain 83 (1999) 379±382
Clinical note
www.elsevier.nl/locate/pain
Supraclavicular glomus tumor, 20 year history of undiagnosed shoulder pain: a case report Ramsis F. Ghaly a,* ,1, Alvin M. Ring b a
Chicago Institute of Neurosurgery and Neuroresearch and Department of Anesthesiology and Pain Management, Cook County Hospital, Chicago, IL, USA b University of Illinois College of Medicine, Chicago and Department of Pathology, Silver Cross Hospital, Joliet, IL, USA Received 25 October 1998; received in revised form 12 March 1999; accepted 12 April 1999
Abstract A long-standing case of severe dysesthesia due to a supraclavicular glomus tumor is presented. Chronic pain caused by a subcutaneous glomus (non-chemodectoma) tumor is rare and usually misdiagnosed. The supraclavicular location, presentation, and coincidence of trauma history are unique in this case. A 62-year-old male complained of 20 years of intractable right shoulder and supraclavicular region pain, which started 6 months after a fall. The pain was unrelieved by repeated and extensive physical therapy, chiropractic manipulation, local steroid injections, and two shoulder operations. The cause of the condition remained undiagnosed and obscure. Local surgical exploration revealed a subcutaneous grayish mass with pathologically proven glomus tumor. Immediate alleviation of the pain and tenderness followed complete resection of the mass. The patient remained free of pain at a 2-year follow-up. Subcutaneous glomus (non-chemodectoma) tumors can occur in unusual sites, and should be considered in chronic regional pain syndromes. Immediate cure is generally achieved by local resection. Pertinent literature is reviewed. q 1999 International Association for the Study of Pain. Published by Elsevier Science B.V. Keywords: Extradigital location; Glomus tumor; Shoulder pain
1. Introduction Glomus tumors are rare but known as benign neoplasm. They are commonly cutaneous painful tumors located in the digits (Drape et al., 1995; Yoshikawa et al., 1996). They arise from the endromyoarterial glomus bodies. A patient may present with local aching pain and exquisite sensitivity to pressure and temperature. Extradigital glomus tumors are uncommon and may represent diagnostic challenge. They can be small, deep-seated and hardly palpable. The following report describes a patient with two-decade history of shoulder pain of unresolved diagnostic dif®culty. This was subsequently diagnosed at surgery. 2. Case report A 62-year-old, right-handed male presented with chronic right shoulder and supraclavicular pain for 20-years. The pain was described as abnormal episodic, sharp aching * Corresponding author. Tel.: 11-773-388-7700; fax: 1773-935-2132. 1 Chicago Institute of Neurosurgery and Neuroresearch, 2515 North Clark Street, Suite 800, Chicago, IL 60614, USA.
pain, with diffuse burning sensation, which was elicited by shoulder movements, especially during gol®ng. The pain score was 8±9 out of 10 on the visual analog scale. In fact, the patient described the severity of his pain as being far worse than what he had experienced during angina attacks and coronary bypass surgery. The pain usually became more evident when resting and at night. There was exquisite hypersensitivity to touch and cold in the whole area. He denied numbness and weakness of the upper extremity. He attributed the onset of the problem to a fall from a hot air balloon. Review of past medical and surgical history contributed nothing further. Examination revealed some wasting of right-shoulder musculature, but normal range-of-motion, and diffuse dysesthesia on palpation. Tenderness was elicited over a spot located on the medial aspect of the right shoulder, with immediate diffuse electric-like shooting pain. There was exquisite hypersensitivity to touch and varied temperature. There was a vague suggestion of a subcutaneous, poorly de®ned lesion less than 1 cm in diameter. An MRI of the cervical spine revealed mild cervical spondylolytic changes. Shoulder imaging studies showed no evidence of obvious shoulder pathology. There was no lesional uptake on the bone scan.
0304-3959/99/$20.00 q 1999 International Association for the Study of Pain. Published by Elsevier Science B.V. PII: S 0304-395 9(99)00094-9
380
R.F. Ghaly, A.M. Ring / Pain 83 (1999) 379±382
3. Intervention The differential diagnosis of the patient's condition were local musculoskeletal and joint disorders and cervical disc disease. Anti-in¯ammatory non-steroidal medications, 72 sessions of physical therapy, and chiropractic manipulations had been tried with no effect. Local cortisone injections offered no relief. Following failure of conservative therapy, the patient underwent two shoulder operations to treat possible clavicula-cromial arthritis. The ®rst surgery was arthroscopic shoulder surgery. The second surgery was performed 1-year later and involved acromioplasty and resection of the distal clavicle. There was no relief of his symptoms after both surgeries.
4. De®nitive surgical procedure A diagnosis of possible subcutaneous nerve tumor (e.g. neuroma) was considered. Under local in®ltration of Lidocaine 1% and intravenous sedation, an incision was made over the tender spot. A subcutaneous grayish, encapsulated ®rm mass, measuring 9 £ 4 £ 4 mm, with tiny whitish attachments, was found and excised in toto. Postoperatively, the patient reported complete pain relief with no dysesthesia or pressure. For the ®rst time in 20-years, he could move the right shoulder freely in any direction without fear of pain. He was able to sleep well and play golf. The patient remains
symptom-free at his 2-year follow-up. The pathological examination revealed a typical glomus tumor. 5. Microscopic description The tumor is encapsulated and consists of clusters of small cells with uniform round to ovoid nuclei and abundant eosinophilic granular to somewhat pale cytoplasm, forming several layers in approximation to the interwoven capillary network. Occasional non-medullated nerve ®bers are present in the tumor especially in the capsular region (Fig. 1). There is no evidence of pleomorphism and only rare mitoses. Silver stains show coarse reticulum around groups of cells and ®ne reticulum around individual cells. Chromogranin and synaptophysin immunostains are negative in the tumor cells. 6. Discussion Glomus tumors (glomangiomas) arise from arteriovenous shunts normally found in the skin especially in the nailbeds, joint regions, and over the scapulae and coccyx (Hall and Odell, 1970; Carroll and Berman, 1972; Drape et al., 1995). The arterial end of these gomera, the Sucquet±Hoyer canal, is surrounded by small, spherical uniform glomus cells which presumably control blood ¯ow by their contractility in response to temperature changes sensed by non-medul-
Fig. 1. Glomus tumor circumscription shown. Capsular region containing blood vessels and non-medullated nerves. (H 1 E, 100 £ ).
R.F. Ghaly, A.M. Ring / Pain 83 (1999) 379±382
381
Fig. 2. Glomus tumor circumscribed nests of uniform cells adjacent to capillaries. Non-medullated nerve crossing through center. (H 1 E, 400 £ ).
lated nerves (Fig. 2). Ultrastructural studies of the glomus cells reveal masses of cytoplasmic ®bers suggestive of derivative smooth muscle cells. The tumors should be distinguished from chemodectomas (paragangliomas) which may also be designated glomus tumor (e.g. glomus jugular tumor). Glomus tumors are most common in the nailbed, (Carroll and Berman, 1972; Drape et al., 1995), but occur on the extremities, trunk, in muscles and joints, and occasionally in viscera (Caughey and Highton, 1966; Hall and Odell, 1970; Smyth, 1971; Chen et al., 1995; Holck and Bredesen, 1996;Yoshikawa et al., 1996). They appear as circumscribed purplish red spots, several millimeters in diameter, and present with characteristic lancinating pain and sensitivity to cold. Histologically, the proportion of glomus cells to vascular channels varies, but generally consist of several rows of peritheliomatous uniform glomus cells, with rare mitoses. If the glomus cells are numerous and overshadow the vascular component, the tumor may be mistaken for a basal cell or sweat gland tumor. A `cell ball-like' peritheliomatous appearance may stimulate a paraganglioma. Generally, the clinical and histologic ®ndings are distinct enough to afford the correct diagnosis. Stains for reticulum, nerve ®bers, factor VIII, keratin, smooth muscle actin, and neuroendocrine granules or ultrastructural studies may be needed occasionally to clarify the diagnosis. The diagnosis is often dif®cult and confusing. Glomus tumors may show sensitivity to temperature variation. Magnetic resonance imaging and ultrasound studies may help localize and visua-
lize the glomus lesion early (Matloub et al., 1992; Drape et al., 1995). Cure and pain alleviation with surgical excision is the rule. Nevertheless, tumor recurrences have been reported. Some authors advocate radiation therapy as a primary modality or as adjuvant for incompletely, resected lesions (Singhal et al., 1993). Reconstructive surgery for local deformity is needed in certain regions such as the nail bed. In conclusion, a deep-seated glomus tumor should be considered in the differential diagnosis of chronic local pain and dysthesia. Magnetic resonance imaging may provide early detection. The tumor has characteristic histologic picture. Immediate and probable permanent cure can be expected after tumor excision. The current case raises the awareness of atypical causes of regional pain syndromes. Acknowledgements The authors thank Jackie Morin for her secretarial help. References Carroll RD, Berman AT. Glomus tumors of the hand; review of the literature and report on twenty-eight cases. J Bone Joint Surg (AM) 1972;54A:691±702. Caughey DE, Highton TC. Glomus tumor of the knee; report of a case. J Bone Joint Surg (BR) 1966;48:134±137. Chen WC, Lee TS, Ru MC, Hsu RC. Glomus tumors of the upper extremity: experience with twelve cases. Chin Med J 1995;55:163±167.
382
R.F. Ghaly, A.M. Ring / Pain 83 (1999) 379±382
Drape SI, Idy Peretti I, Goetmann S, et al. Subungual glomus tumours: Evaluation with MR imaging. Radiology 1995;195:507. Hall RM, Odell JE. An unusually large glomangioma simulating osteoarthritis of the hip. J Am Med Assoc 1970;214:1562. Holck S, Bredesen SL. Solid glomus tumor presenting as an axillary mass: Report of a case with morphologic study, including cytologic characteristics. Acta cytologica 1996;40:555±562. Matloub HS, Muoneke VN, Prevel CD, Sanger JR, Yousif NJ. Glomus tumor imaging: Use of MRI for localization of occult lesions. J Hand Surg Am 1992;17:472±475.
Singhal S, Sharma S, Dixit S, Sundarsan DE, Chander S, Rath GR. Primary radiation therapy in the management of glomus tumors. Indian J Cancer 1993;30:120±124. Smyth M. Glomus-cell tumors in the lower extremity; report of two cases. J Bone Joint Surg (Am) 1971;53:157±159. Yoshikawa G, Murakami M, Ishizawa M, Matsumoto R, Hukuda S. Glomus tumor of the musculotendonous junction of the rotator cuff: a case report. Clin Ortho Related Res 1996;326:250±253.
Clinical note
www.elsevier.nl/locate/pain
Supraclavicular glomus tumor, 20 year history of undiagnosed shoulder pain: a case report Ramsis F. Ghaly a,* ,1, Alvin M. Ring b a
Chicago Institute of Neurosurgery and Neuroresearch and Department of Anesthesiology and Pain Management, Cook County Hospital, Chicago, IL, USA b University of Illinois College of Medicine, Chicago and Department of Pathology, Silver Cross Hospital, Joliet, IL, USA Received 25 October 1998; received in revised form 12 March 1999; accepted 12 April 1999
Abstract A long-standing case of severe dysesthesia due to a supraclavicular glomus tumor is presented. Chronic pain caused by a subcutaneous glomus (non-chemodectoma) tumor is rare and usually misdiagnosed. The supraclavicular location, presentation, and coincidence of trauma history are unique in this case. A 62-year-old male complained of 20 years of intractable right shoulder and supraclavicular region pain, which started 6 months after a fall. The pain was unrelieved by repeated and extensive physical therapy, chiropractic manipulation, local steroid injections, and two shoulder operations. The cause of the condition remained undiagnosed and obscure. Local surgical exploration revealed a subcutaneous grayish mass with pathologically proven glomus tumor. Immediate alleviation of the pain and tenderness followed complete resection of the mass. The patient remained free of pain at a 2-year follow-up. Subcutaneous glomus (non-chemodectoma) tumors can occur in unusual sites, and should be considered in chronic regional pain syndromes. Immediate cure is generally achieved by local resection. Pertinent literature is reviewed. q 1999 International Association for the Study of Pain. Published by Elsevier Science B.V. Keywords: Extradigital location; Glomus tumor; Shoulder pain
1. Introduction Glomus tumors are rare but known as benign neoplasm. They are commonly cutaneous painful tumors located in the digits (Drape et al., 1995; Yoshikawa et al., 1996). They arise from the endromyoarterial glomus bodies. A patient may present with local aching pain and exquisite sensitivity to pressure and temperature. Extradigital glomus tumors are uncommon and may represent diagnostic challenge. They can be small, deep-seated and hardly palpable. The following report describes a patient with two-decade history of shoulder pain of unresolved diagnostic dif®culty. This was subsequently diagnosed at surgery. 2. Case report A 62-year-old, right-handed male presented with chronic right shoulder and supraclavicular pain for 20-years. The pain was described as abnormal episodic, sharp aching * Corresponding author. Tel.: 11-773-388-7700; fax: 1773-935-2132. 1 Chicago Institute of Neurosurgery and Neuroresearch, 2515 North Clark Street, Suite 800, Chicago, IL 60614, USA.
pain, with diffuse burning sensation, which was elicited by shoulder movements, especially during gol®ng. The pain score was 8±9 out of 10 on the visual analog scale. In fact, the patient described the severity of his pain as being far worse than what he had experienced during angina attacks and coronary bypass surgery. The pain usually became more evident when resting and at night. There was exquisite hypersensitivity to touch and cold in the whole area. He denied numbness and weakness of the upper extremity. He attributed the onset of the problem to a fall from a hot air balloon. Review of past medical and surgical history contributed nothing further. Examination revealed some wasting of right-shoulder musculature, but normal range-of-motion, and diffuse dysesthesia on palpation. Tenderness was elicited over a spot located on the medial aspect of the right shoulder, with immediate diffuse electric-like shooting pain. There was exquisite hypersensitivity to touch and varied temperature. There was a vague suggestion of a subcutaneous, poorly de®ned lesion less than 1 cm in diameter. An MRI of the cervical spine revealed mild cervical spondylolytic changes. Shoulder imaging studies showed no evidence of obvious shoulder pathology. There was no lesional uptake on the bone scan.
0304-3959/99/$20.00 q 1999 International Association for the Study of Pain. Published by Elsevier Science B.V. PII: S 0304-395 9(99)00094-9
380
R.F. Ghaly, A.M. Ring / Pain 83 (1999) 379±382
3. Intervention The differential diagnosis of the patient's condition were local musculoskeletal and joint disorders and cervical disc disease. Anti-in¯ammatory non-steroidal medications, 72 sessions of physical therapy, and chiropractic manipulations had been tried with no effect. Local cortisone injections offered no relief. Following failure of conservative therapy, the patient underwent two shoulder operations to treat possible clavicula-cromial arthritis. The ®rst surgery was arthroscopic shoulder surgery. The second surgery was performed 1-year later and involved acromioplasty and resection of the distal clavicle. There was no relief of his symptoms after both surgeries.
4. De®nitive surgical procedure A diagnosis of possible subcutaneous nerve tumor (e.g. neuroma) was considered. Under local in®ltration of Lidocaine 1% and intravenous sedation, an incision was made over the tender spot. A subcutaneous grayish, encapsulated ®rm mass, measuring 9 £ 4 £ 4 mm, with tiny whitish attachments, was found and excised in toto. Postoperatively, the patient reported complete pain relief with no dysesthesia or pressure. For the ®rst time in 20-years, he could move the right shoulder freely in any direction without fear of pain. He was able to sleep well and play golf. The patient remains
symptom-free at his 2-year follow-up. The pathological examination revealed a typical glomus tumor. 5. Microscopic description The tumor is encapsulated and consists of clusters of small cells with uniform round to ovoid nuclei and abundant eosinophilic granular to somewhat pale cytoplasm, forming several layers in approximation to the interwoven capillary network. Occasional non-medullated nerve ®bers are present in the tumor especially in the capsular region (Fig. 1). There is no evidence of pleomorphism and only rare mitoses. Silver stains show coarse reticulum around groups of cells and ®ne reticulum around individual cells. Chromogranin and synaptophysin immunostains are negative in the tumor cells. 6. Discussion Glomus tumors (glomangiomas) arise from arteriovenous shunts normally found in the skin especially in the nailbeds, joint regions, and over the scapulae and coccyx (Hall and Odell, 1970; Carroll and Berman, 1972; Drape et al., 1995). The arterial end of these gomera, the Sucquet±Hoyer canal, is surrounded by small, spherical uniform glomus cells which presumably control blood ¯ow by their contractility in response to temperature changes sensed by non-medul-
Fig. 1. Glomus tumor circumscription shown. Capsular region containing blood vessels and non-medullated nerves. (H 1 E, 100 £ ).
R.F. Ghaly, A.M. Ring / Pain 83 (1999) 379±382
381
Fig. 2. Glomus tumor circumscribed nests of uniform cells adjacent to capillaries. Non-medullated nerve crossing through center. (H 1 E, 400 £ ).
lated nerves (Fig. 2). Ultrastructural studies of the glomus cells reveal masses of cytoplasmic ®bers suggestive of derivative smooth muscle cells. The tumors should be distinguished from chemodectomas (paragangliomas) which may also be designated glomus tumor (e.g. glomus jugular tumor). Glomus tumors are most common in the nailbed, (Carroll and Berman, 1972; Drape et al., 1995), but occur on the extremities, trunk, in muscles and joints, and occasionally in viscera (Caughey and Highton, 1966; Hall and Odell, 1970; Smyth, 1971; Chen et al., 1995; Holck and Bredesen, 1996;Yoshikawa et al., 1996). They appear as circumscribed purplish red spots, several millimeters in diameter, and present with characteristic lancinating pain and sensitivity to cold. Histologically, the proportion of glomus cells to vascular channels varies, but generally consist of several rows of peritheliomatous uniform glomus cells, with rare mitoses. If the glomus cells are numerous and overshadow the vascular component, the tumor may be mistaken for a basal cell or sweat gland tumor. A `cell ball-like' peritheliomatous appearance may stimulate a paraganglioma. Generally, the clinical and histologic ®ndings are distinct enough to afford the correct diagnosis. Stains for reticulum, nerve ®bers, factor VIII, keratin, smooth muscle actin, and neuroendocrine granules or ultrastructural studies may be needed occasionally to clarify the diagnosis. The diagnosis is often dif®cult and confusing. Glomus tumors may show sensitivity to temperature variation. Magnetic resonance imaging and ultrasound studies may help localize and visua-
lize the glomus lesion early (Matloub et al., 1992; Drape et al., 1995). Cure and pain alleviation with surgical excision is the rule. Nevertheless, tumor recurrences have been reported. Some authors advocate radiation therapy as a primary modality or as adjuvant for incompletely, resected lesions (Singhal et al., 1993). Reconstructive surgery for local deformity is needed in certain regions such as the nail bed. In conclusion, a deep-seated glomus tumor should be considered in the differential diagnosis of chronic local pain and dysthesia. Magnetic resonance imaging may provide early detection. The tumor has characteristic histologic picture. Immediate and probable permanent cure can be expected after tumor excision. The current case raises the awareness of atypical causes of regional pain syndromes. Acknowledgements The authors thank Jackie Morin for her secretarial help. References Carroll RD, Berman AT. Glomus tumors of the hand; review of the literature and report on twenty-eight cases. J Bone Joint Surg (AM) 1972;54A:691±702. Caughey DE, Highton TC. Glomus tumor of the knee; report of a case. J Bone Joint Surg (BR) 1966;48:134±137. Chen WC, Lee TS, Ru MC, Hsu RC. Glomus tumors of the upper extremity: experience with twelve cases. Chin Med J 1995;55:163±167.
382
R.F. Ghaly, A.M. Ring / Pain 83 (1999) 379±382
Drape SI, Idy Peretti I, Goetmann S, et al. Subungual glomus tumours: Evaluation with MR imaging. Radiology 1995;195:507. Hall RM, Odell JE. An unusually large glomangioma simulating osteoarthritis of the hip. J Am Med Assoc 1970;214:1562. Holck S, Bredesen SL. Solid glomus tumor presenting as an axillary mass: Report of a case with morphologic study, including cytologic characteristics. Acta cytologica 1996;40:555±562. Matloub HS, Muoneke VN, Prevel CD, Sanger JR, Yousif NJ. Glomus tumor imaging: Use of MRI for localization of occult lesions. J Hand Surg Am 1992;17:472±475.
Singhal S, Sharma S, Dixit S, Sundarsan DE, Chander S, Rath GR. Primary radiation therapy in the management of glomus tumors. Indian J Cancer 1993;30:120±124. Smyth M. Glomus-cell tumors in the lower extremity; report of two cases. J Bone Joint Surg (Am) 1971;53:157±159. Yoshikawa G, Murakami M, Ishizawa M, Matsumoto R, Hukuda S. Glomus tumor of the musculotendonous junction of the rotator cuff: a case report. Clin Ortho Related Res 1996;326:250±253.