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| CASE REPORT |
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| Year : 2012 | Volume
: 39
| Issue : 3 | Page : 152-154 |
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An unusual case of extra-abdominal desmoid tumour of the finger
V Navaneeth, R Vaithianathan, R Santhanam
Department of Surgery, Mahatma Gandhi Medical College and Research Institute, Pondicherry, India
| Date of Web Publication | 11-Jan-2013 |
Correspondence Address:
R Vaithianathan
Flat 5, Nallam Mansion, 49 Anna Nagar Main Road, Anna Nagar, Pondicherry - 605 005
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0974-5009.105926
Extra-abdominal desmoid tumour is a rare tumour and involvement of the finger is extremely rare. These are generally non-metastasizing lesion of mesenchymal origin composed of fibrous tissue with locally aggressive proliferative behaviour. We report a case of extra-abdominal desmoid in a sixty year year old lady involving the right index finger. The swelling was slowly increasing in size over a period of eleven years and involved almost the entire length of the finger. A disarticulation at the second metacarpophalangeal joint was done and histopathological examination confirmed it to be a desmoid tumour. Keywords: Desmoid tumour, extra-abdominal desmoid, excision
How to cite this article:
Navaneeth V, Vaithianathan R, Santhanam R. An unusual case of extra-abdominal desmoid tumour of the finger. J Sci Soc 2012;39:152-4 |
| Introduction | |  |
Desmoid tumours are rare, benign, but locally aggressive clonal fibroblastic proliferations arising from the deep soft tissues. These are characterized by infiltrative growth with a tendency for local recurrence, but without any risk for distant spread. [1] This tumour accounts for 0.03% of all tumours and 3% of soft-tissue tumours with annual incidence of 2-4 cases per million. [2] Based on the aetiology, desmoid tumours are classified into three biologic groups: Sporadic, those associated with familial adenomatous polyposis syndrome and the familial group. Based on the anatomical location they are divided into intra-abdominal, abdominal wall desmoid and extra-abdominal type. Extra-abdominal desmoid is a very rare tumour and involvement of finger is extremely unusual and only very few cases have been reported in the literature. [3]
| Case Report | | |
A sixty year old woman, working at a quarry, presented with a swelling arising from her right index finger of eleven years duration. There was history of recent trauma to this finger while at work and this led to ulceration at summit of the lump. Examination revealed an ulcerated, fleshy tumour of size 9 cm × 7 cm, involving the distal two thirds of the index finger [Figure 1]. Radiography of the hand showed no bony involvement [Figure 2]. Core biopsy of the swelling revealed features of benign spindle cell tumour.
A disarticulation at the second metacarpophalangeal joint was carried out as the finger was painful and non-functional. The histopathological examination showed features of desmoid tumour with proliferating stellate to spindle cells arranged in whorling patterns of fibroblast and myofibroblasts [Figure 3]. There were no nuclear atypia and hyperchromasia and the cells were strongly positive for vimentin.
| Discussion | | |
Desmoid tumour is also known as well differentiated fibroblastic tumour arising from the connective tissues of muscles, fascia, aponeurosis or periosteum. [4] It is a locally aggressive tumour, well known to invade nearby structures and can cause compression of the intestines, ureters, other viscera, major vessels and nerves. [2] Considering their rarity in clinical practice, desmoid tumours are often wrongly diagnosed. These can present with typical clinical features of soft tissue tumours and can mimic fibrosarcomas, synovial sarcomas or neurofibromas. A definitive diagnosis will be aided by a complete histopathological assessment of the tumour. The microscopic aspect is typically that of uniform bundles of spindle-shaped fibroblasts and collagen depositions with an acellular centre and absence of pseudocapsule. Macrophages, giant cells and lymphocytes are present peripherally. [5]
Currently, multidisciplinary approach is necessary for the management of desmoid fibromatosis. A surgical approach with complete excision and clear margins is the only effective method of cure, with the lowest incidence of recurrence. Amputation is reserved for refractory cases and when the extremity or the digit is non-functional or chronically painful.
Many non surgical options like chemotherapy, tamoxifen, imatinib, interferon, anti-inflammatory agents and theophylline have all been tried with some success in non resectable cases. [6] These tumours express oestrogen receptors and hence treatment with tamoxifen, gonadotropin-releasing hormone agonists or aromatase inhibitors is known to produce regression in some patients. [7] Surgical resection may be contraindicated in patients with recurrent extra-abdominal desmoid tumours. Here, a combination regimen of methotrexate with either vinblastine or vinorelbine has been found effective for tumour control. Radiation therapy has also been employed to manage recurrent disease or as primary therapy to avoid mutilating surgical resection. [6],[8]
| Conclusion | | |
In conclusion, the occurrence of desmoid tumours in the limbs is extremely uncommon. They are commonly mistaken for other soft tissue tumours due their clinical or imaging findings. One should always rule out desmoid tumour for swellings arising from the extremities, especially with indolent clinical presentation. The correct diagnosis should be established based on histological confirmation.
| References | | |
| 1. | Reitamo JJ, Scheinin TM, Hayry P. The desmoid syndrome. New aspects in the causes, pathogenesis and treatment of the desmoid tumour. Am J Surg 1986;151:230-7 
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| 2. | Zampieri N, Cecchetto M, Zorzi MG, Pietrobelli A, Ottolenghi A, Camoglio F. An unusual case of extra-abdominal desmoid tumour. Eur J Cancer Care 2010;19:410-2.
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| 4. | Deyrup AT, Tretiakova M, Montag AG. Estrogen receptor beta-expression in extra-abdominal fibromatoses: An analysis of 40 cases. Cancer 2006;106:208-13.
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| 5. | Ayala AG, Ro JY, Goepfert H, Cangir A, Khorsand J, Flake G. Desmoid fibromatosis: A clinicopathologic study of 25 children. Semin Diagn Pathol 1986;3:138-50.
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| 6. | Escobar C, Munker R, Thomas JO, Li BD, Burton GV. Update on desmoid tumours. Ann Oncol. 2012;23:562-9.
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| 7. | Wilcken N, Tattersall MH. Endocrine therapy for desmoid tumours. Cancer 1991;68:1384-8.
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| 8. | Pignatti G, Barbanti-Brodano G, Ferrari D, Gherlinzoni F, Bretoni F, Bacchini P, et al. Extra-abdominal desmoid tumour. A study of 83 cases. Clin Orthop Relat Res. 2000;375:207-13.
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[Figure 1], [Figure 2], [Figure 3]
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