The Open Orthopaedics Journal




ISSN: 1874-3250 ― Volume 13, 2019

Synovial Angioma of the FDP Flexor Sheath: A Rare Cause of Carpal Tunnel Syndrome



Robert George*, Kenneth Lee
Royal Prince Alfred Hospital, Camperdown, NSW, Australia

Abstract

It has been previously noted that synovial haemangiomas in the hand and wrist are very rare pathological entities. We report the case of a 34-year-old right hand dominant male who presented to his general practitioner with an enlarging left volar wrist/ palmar mass, who further developed symptoms consistent with carpal tunnel syndrome. An MRI scan subsequently confirmed a large, complex mass with area of necrosis and peripheral enhancement. The rate of mass growth and radiological features raised the possibility of a soft tissue malignancy, and the gentleman was urgently referred to our unit for surgical exploration and removal of tumour. Surgical exploration demonstrated a tan-coloured soft tissue mass on the ulnar aspect of the median nerve. It appeared to arise from, and marginally infiltrated, the tendon sheath of the FDP tendon to the ring finger and the lumbrical muscle of the fourth ray; the distal and proximal extent of the tumour was difficult to define due to the diffuse growth of the tumour. Resection was achieved with macroscopic margins, with excellent functional recovery immediately and at 6 month follow-up. Histological analysis was consistent with a synovial haemangioma, comprising of numerous thin-walled blood vessels with a central cystic cavity containing blood and fibrin. Our case further demonstrates the diagnostic challenges posed by compressive neuropathy due to soft tissue masses, even with thorough clinical and radiological assessment. In the context of a rapidly growing tumour, malignancy must always be suspected and might highlight a role for pre-operative biopsy.

Keywords: Carpal tunnel, synovial angioma, median nerve compression, tumour.


Article Information


Identifiers and Pagination:

Year: 2013
Volume: 7
First Page: 72
Last Page: 74
Publisher Id: TOORTHJ-7-72
DOI: 10.2174/1874325001307010072

Article History:

Received Date: 22/11/2012
Revision Received Date: 22/2/2013
Acceptance Date: 25/2/2013
Electronic publication date: 19/3/2013
Collection year: 2013

Article Metrics:

CrossRef Citations:
0

Total Statistics:

Full-Text HTML Views: 874
Abstract HTML Views: 625
PDF Downloads: 155
Total Views/Downloads: 1654

Unique Statistics:

Full-Text HTML Views: 605
Abstract HTML Views: 373
PDF Downloads: 109
Total Views/Downloads: 1087
Geographical View

© George and Lee; Licensee Bentham Open.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.5/) which permits unrestrictive use, distribution, and reproduction in any medium, provided the original work is properly cited.


* Address correspondence to this author at the P.O. Box 309, Crows Nest, 2065, Australia; Tel: + 61 420 982 306; E-mail: dr.robertg@gmail.com




INTRODUCTION

Carpal Tunnel Syndrome (CTS) is a common disorder in both primary care and specialist practice [1Atroshi I, Gummesson C, Johnsson R, et al. Prevalence of carpal tunnel syndrome in a general population JAMA 1999; 282: 153-8.], usually caused by a chronic tenosynovitis of the transverse carpal ligament (TCL) (or flexor retinaculum). However, macroscopically demonstrable space occupying lesions within the carpal tunnel are also a well described, though relatively uncommon cause [2Stevens J, Beard C, Ofallon W, et al. conditions associated with carpal-tunnel syndrome Mayo Clin Proc 1992; 67: 541-8.]. We describe a rare case of synovial haemangioma arising from the flexor digitorum profundus tendon sheath presenting as a rapidly enlarging wrist mass with median nerve neuropathy. Due to its alarming presentation history, atypical pathology and complex anatomical position, it posed a difficult diagnostic problem.

CASE REPORT

A 34 year old right hand-dominant male, who works as a chef, presented with a 4 month history of left wrist mass. It had increased rapidly in size during the final month. The patient also began to experience numbness of his left thumb, index and middle fingers. He was normally well and not on regular medications. There was no family history of note.

An ultrasound was initially performed which showed a large, complex cystic structure within the carpal tunnel. A subsequent MRI revealed a heterogeneous, well defined mass with fluid and solid components. The mass was located middle and ring fingers. It appeared to arise from the tendon sheath of the middle and ring finger flexors, but was not between the superficial and deep flexor tendon sheaths of the invading the tendons. It extended distally to the carpal tunnel, with overall dimensions of 19 x 24 x 38 mm. It showed peripheral enhancement and areas of necrosis, and there was no flow void (Fig. 1). The median nerve had a normal appearance. In light of the clinical history and radiological features, a malignant lesion such a synovial sarcoma, was considered the likely provisional diagnosis.

Fig. (1)

T1 weighted MRI of a 19 x 24 x 38 mm complex mass within Carpal Tunnel.



The patient was referred to our unit for urgent review. On examination he had a mobile and palpable mass which moved from the mid palm to the volar wrist region with flexion and extension of his wrist. The mass was not adherent to overlying skin. He had full power in his left hand and there was no thenar muscle wasting. He had decreased sensation in his thumb, index and middle finger, and Tinel’s test was negative.

He underwent surgical exploration and removal of tumour within one week of the consultation. An extended carpal tunnel incision was made (Fig. 2), from the mid palm to the volar wrist. After the TCL was divided, a large tan coloured soft-tissue tumor was visible on the ulnar aspect of the median nerve. It appeared to arise from the deep flexor tendon sheath of the ring finger, extending distally to the lumbrical muscle of the 4th ray, and it infiltrated these structures only minimally. A persistent median artery was observed overlying the median nerve, which did not appear to be involved by the tumour. The tumour was resected with macroscopic margins. Post-operative recovery was uneventful and the patient reported immediate relief of compressive median nerve symptoms.

Fig. (2)

Intraoperative view of case; a tan-coloured soft tissue tumour immediately adjacent to the Median nerve was identified and resected.



Histological analysis was consistent with a synovial haemangioma, comprising of numerous thin-walled blood vessels with a central cystic cavity filled with blood and fibrin (Fig. 3). There was no evidence of malignancy.

Fig. (3)

Histological analysis of the specimen, showing pathological hallmarks of a synovial angioma.



A three month follow-up confirmed complete resolution of his neurosensory symptoms and showed a well healing scar.

DISCUSSION

The overwhelming majority of cases of CTS are caused by a chronic, non-specific tenosynovitis of the TCL [2Stevens J, Beard C, Ofallon W, et al. conditions associated with carpal-tunnel syndrome Mayo Clin Proc 1992; 67: 541-8., 3De Krom M, Kester A, Knipschild J, et al. Risk factors for carpal tunnel syndrome Am J Epidemiol 1990; 132: 1102-0.]. Histological analysis of involved tissues is often normal and there are no features which might be considered pathognomonic [4Nakamichi KI, Tachibana S. Histology of the transverse carpal ligament and flexor tenosynovium in idiopathic carpal tunnel syndrome J Hand Surg Am 1998; 23: 1015-24.]. Whilst rare, space occupying lesions (SOL) within or adjacent to the carpal tunnel are nevertheless a recognised cause of CTS. Several types of soft tissue masses have been implicated in compressive neuropathy of the median nerve, such as aneurysms, vascular malformations, tuberculomas, neurofibromas, lipomas, ganglia and gouty tophi [2Stevens J, Beard C, Ofallon W, et al. conditions associated with carpal-tunnel syndrome Mayo Clin Proc 1992; 67: 541-8., 5Kang HJ, Jung SH, Yoon HK, Hahn SB, Kim SJ. Carpal tunnel syndrome caused by space occupying lesions Yonsei Med J 2009; 50(2): 257-61.]. Irrespective of the primary aetiology, electrophysiological studies suggest that the underlying pathophysiology relates to compression of the vasa nervosum of the nerve [6Sunderland S. The nerve lesion in the carpal tunnel syndrome J Neurol Neurosurg Psychiatr 1976; 1: 615-25.].

Synovial angiomas are benign tumours characterised by a proliferation of blood vessels arising from a synovium-lined surface [7Calonje E. Haemangiomas In: Christopher DM, Fletcher K, Krishnan U, Mertens F, Eds. Pathology & genetics: tumours of soft tissue & bone. Lyon: IARC Press 2002; pp. 156-7.]. Unlike the common cutaneous form [8Rubin E, Farber JL. Pathology. Philadelphia: Lippincott-Raven 1999; pp. 89-93.], synovial angiomas are rarely encountered pathological entities. They have only been described in isolated reports and small case series. The overwhelming majority occur in the knee joint, however, they have also been described to occur in the upper limb, such as the elbow joint, wrist joint and even interphalangeal joints of the fingers [9Greenspan A, Azouz EM, Matthews J, et al. Synovial hemangioma: imaging features in eight histologically proven cases, review of the literature, and differential diagnosis Skeletal Radiol 1995; 24: 583-90.-11Talwalkar S, Haytion M, Stilwell J, et al. Tenosynovial haemangioma of the finger Acta Orthop Belg 2005; 71: 618-21.]. Synovial angiomas arising from the upper extremity tendon sheaths are exceedingly rare [11Talwalkar S, Haytion M, Stilwell J, et al. Tenosynovial haemangioma of the finger Acta Orthop Belg 2005; 71: 618-21.-13Weinzweig J, Watson HK, Genter BE. Hemangioma of the extensor pollicis brevis in the first dorsal compartment: an unusual cause of bilateral de Quervain's disease J Hand Surg 1996; 21: 256-8.]. To date, only one case of synovial angioma arising from a tendon sheath within the carpal tunnel has been reported [14Kim JY, Sung JH, Lee S. A haemangioma of the flexor tendon sheath causing carpal tunnel syndrome J Hand Surg Eur Vol 2010; 35: 73-4.].

Previous reports suggest that surgical outcome can be optimised with accurate preoperative assessment and diagnosis. However, this is difficult to achieve when encountering rarely encountered soft tissue tumours (eg synovial angiomas) arising from anatomically complex spaces (such as the carpal tunnel) [5Kang HJ, Jung SH, Yoon HK, Hahn SB, Kim SJ. Carpal tunnel syndrome caused by space occupying lesions Yonsei Med J 2009; 50(2): 257-61.]. Apart from the presence of a mass, clinical history and examination are often non-specific. Adjunctive imaging, such as MRI, can be helpful but often non-specific for soft –tissue tumour diagnosis [15Chalidis B, Dimitriou C. Carpal tunnel syndrome due to an atypical deep soft tissue leiomyoma: The risk of misdiagnosis and mismanagement World J Surg Oncol 2007; 5: 92.], especially when the mass is a haemangioma. Our case contained the radiographic hallmarks of a potentially malignant neoplasm – rapidly growing mass with complex structure, areas of focal necrosis and peripheral enhancement, and lacked the flowed voids normally associated with benign vascular lesions [16The J, WHiteley G. MRI of soft tissue masses of the hand and wrist Br J Radiol 2007; 80: 47-63.]. Further radiological clarification might have been obtained by digital subtraction angiography, however this requires an index of suspicion that the tumor is of vascular origin [9Greenspan A, Azouz EM, Matthews J, et al. Synovial hemangioma: imaging features in eight histologically proven cases, review of the literature, and differential diagnosis Skeletal Radiol 1995; 24: 583-90.].

Our case further demonstrates the diagnostic challenges posed by compressive neuropathy due to soft tissue masses. In the context of a rapidly growing tumour, malignancy must always be suspected. A clear pre-operative diagnosis optimises patient’s safety and surgical outcome. Given the difficulty in assessing soft tissue tumours of the carpal tunnel, radiological correlation should be sought from a unit with an interest in soft tissue neoplasms to supplement thorough clinical assessment. Moreover, radiologic guided biopsy could further aid pre-operative diagnosis and management planning.

CONFLICT OF INTEREST

The authors confirm that this article content has no conflict of interest.

ACKNOWLEDGEMENTS

We would like to thank Prof Soon Lee & Dr Yvonne Bogun from the Department of Anatomical Pathology, Royal Prince Alfred Hospital.

REFERENCES

[1] Atroshi I, Gummesson C, Johnsson R, et al. Prevalence of carpal tunnel syndrome in a general population JAMA 1999; 282: 153-8.
[2] Stevens J, Beard C, Ofallon W, et al. conditions associated with carpal-tunnel syndrome Mayo Clin Proc 1992; 67: 541-8.
[3] De Krom M, Kester A, Knipschild J, et al. Risk factors for carpal tunnel syndrome Am J Epidemiol 1990; 132: 1102-0.
[4] Nakamichi KI, Tachibana S. Histology of the transverse carpal ligament and flexor tenosynovium in idiopathic carpal tunnel syndrome J Hand Surg Am 1998; 23: 1015-24.
[5] Kang HJ, Jung SH, Yoon HK, Hahn SB, Kim SJ. Carpal tunnel syndrome caused by space occupying lesions Yonsei Med J 2009; 50(2): 257-61.
[6] Sunderland S. The nerve lesion in the carpal tunnel syndrome J Neurol Neurosurg Psychiatr 1976; 1: 615-25.
[7] Calonje E. Haemangiomas In: Christopher DM, Fletcher K, Krishnan U, Mertens F, Eds. Pathology & genetics: tumours of soft tissue & bone. Lyon: IARC Press 2002; pp. 156-7.
[8] Rubin E, Farber JL. Pathology. Philadelphia: Lippincott-Raven 1999; pp. 89-93.
[9] Greenspan A, Azouz EM, Matthews J, et al. Synovial hemangioma: imaging features in eight histologically proven cases, review of the literature, and differential diagnosis Skeletal Radiol 1995; 24: 583-90.
[10] Nelson MC, Stull MA, Teitelbaum GP, et al. Magnetic resonance imaging of peripheral soft tissue hemangiomas Skeletal Radiol 1990; 19: 477-82.
[11] Talwalkar S, Haytion M, Stilwell J, et al. Tenosynovial haemangioma of the finger Acta Orthop Belg 2005; 71: 618-21.
[12] Waddell GF. A haemangioma involving tendons J Bone Joint Surg Am 1967; 49(1): 138-41.
[13] Weinzweig J, Watson HK, Genter BE. Hemangioma of the extensor pollicis brevis in the first dorsal compartment: an unusual cause of bilateral de Quervain's disease J Hand Surg 1996; 21: 256-8.
[14] Kim JY, Sung JH, Lee S. A haemangioma of the flexor tendon sheath causing carpal tunnel syndrome J Hand Surg Eur Vol 2010; 35: 73-4.
[15] Chalidis B, Dimitriou C. Carpal tunnel syndrome due to an atypical deep soft tissue leiomyoma: The risk of misdiagnosis and mismanagement World J Surg Oncol 2007; 5: 92.
[16] The J, WHiteley G. MRI of soft tissue masses of the hand and wrist Br J Radiol 2007; 80: 47-63.

Endorsements



"Open access will revolutionize 21st century knowledge work and accelerate the diffusion of ideas and evidence that support just in time learning and the evolution of thinking in a number of disciplines."


Daniel Pesut
(Indiana University School of Nursing, USA)

"It is important that students and researchers from all over the world can have easy access to relevant, high-standard and timely scientific information. This is exactly what Open Access Journals provide and this is the reason why I support this endeavor."


Jacques Descotes
(Centre Antipoison-Centre de Pharmacovigilance, France)

"Publishing research articles is the key for future scientific progress. Open Access publishing is therefore of utmost importance for wider dissemination of information, and will help serving the best interest of the scientific community."


Patrice Talaga
(UCB S.A., Belgium)

"Open access journals are a novel concept in the medical literature. They offer accessible information to a wide variety of individuals, including physicians, medical students, clinical investigators, and the general public. They are an outstanding source of medical and scientific information."


Jeffrey M. Weinberg
(St. Luke's-Roosevelt Hospital Center, USA)

"Open access journals are extremely useful for graduate students, investigators and all other interested persons to read important scientific articles and subscribe scientific journals. Indeed, the research articles span a wide range of area and of high quality. This is specially a must for researchers belonging to institutions with limited library facility and funding to subscribe scientific journals."


Debomoy K. Lahiri
(Indiana University School of Medicine, USA)

"Open access journals represent a major break-through in publishing. They provide easy access to the latest research on a wide variety of issues. Relevant and timely articles are made available in a fraction of the time taken by more conventional publishers. Articles are of uniformly high quality and written by the world's leading authorities."


Robert Looney
(Naval Postgraduate School, USA)

"Open access journals have transformed the way scientific data is published and disseminated: particularly, whilst ensuring a high quality standard and transparency in the editorial process, they have increased the access to the scientific literature by those researchers that have limited library support or that are working on small budgets."


Richard Reithinger
(Westat, USA)

"Not only do open access journals greatly improve the access to high quality information for scientists in the developing world, it also provides extra exposure for our papers."


J. Ferwerda
(University of Oxford, UK)

"Open Access 'Chemistry' Journals allow the dissemination of knowledge at your finger tips without paying for the scientific content."


Sean L. Kitson
(Almac Sciences, Northern Ireland)

"In principle, all scientific journals should have open access, as should be science itself. Open access journals are very helpful for students, researchers and the general public including people from institutions which do not have library or cannot afford to subscribe scientific journals. The articles are high standard and cover a wide area."


Hubert Wolterbeek
(Delft University of Technology, The Netherlands)

"The widest possible diffusion of information is critical for the advancement of science. In this perspective, open access journals are instrumental in fostering researches and achievements."


Alessandro Laviano
(Sapienza - University of Rome, Italy)

"Open access journals are very useful for all scientists as they can have quick information in the different fields of science."


Philippe Hernigou
(Paris University, France)

"There are many scientists who can not afford the rather expensive subscriptions to scientific journals. Open access journals offer a good alternative for free access to good quality scientific information."


Fidel Toldrá
(Instituto de Agroquimica y Tecnologia de Alimentos, Spain)

"Open access journals have become a fundamental tool for students, researchers, patients and the general public. Many people from institutions which do not have library or cannot afford to subscribe scientific journals benefit of them on a daily basis. The articles are among the best and cover most scientific areas."


M. Bendandi
(University Clinic of Navarre, Spain)

"These journals provide researchers with a platform for rapid, open access scientific communication. The articles are of high quality and broad scope."


Peter Chiba
(University of Vienna, Austria)

"Open access journals are probably one of the most important contributions to promote and diffuse science worldwide."


Jaime Sampaio
(University of Trás-os-Montes e Alto Douro, Portugal)

"Open access journals make up a new and rather revolutionary way to scientific publication. This option opens several quite interesting possibilities to disseminate openly and freely new knowledge and even to facilitate interpersonal communication among scientists."


Eduardo A. Castro
(INIFTA, Argentina)

"Open access journals are freely available online throughout the world, for you to read, download, copy, distribute, and use. The articles published in the open access journals are high quality and cover a wide range of fields."


Kenji Hashimoto
(Chiba University, Japan)

"Open Access journals offer an innovative and efficient way of publication for academics and professionals in a wide range of disciplines. The papers published are of high quality after rigorous peer review and they are Indexed in: major international databases. I read Open Access journals to keep abreast of the recent development in my field of study."


Daniel Shek
(Chinese University of Hong Kong, Hong Kong)

"It is a modern trend for publishers to establish open access journals. Researchers, faculty members, and students will be greatly benefited by the new journals of Bentham Science Publishers Ltd. in this category."


Jih Ru Hwu
(National Central University, Taiwan)


Browse Contents



Webmaster Contact: info@benthamopen.net
Copyright © 2019 Bentham Open