ISPUB.com / IJPA/13/3/14262
  • Author/Editor Login
  • Registration
  • Facebook
  • Google Plus

ISPUB.com

Internet
Scientific
Publications

  • Home
  • Journals
  • Latest Articles
  • Disclaimers
  • Article Submissions
  • Contact
  • Help
  • The Internet Journal of Pathology
  • Volume 13
  • Number 3

Original Article

Extraabdominal-Desmoid-Type Fibromatosis In The Index Finger: A Rare Case Report

A Jain, D Kotasthane, G Koteeswaran

Keywords

aggressive fibromatosis, extra abdominal desmoid tumor, index finger

Citation

A Jain, D Kotasthane, G Koteeswaran. Extraabdominal-Desmoid-Type Fibromatosis In The Index Finger: A Rare Case Report. The Internet Journal of Pathology. 2012 Volume 13 Number 3.

Abstract

Desmoid tumors are rare, accounting for 0.03% of all neoplasms and with an estimated incidence rate of 2-4 per million per year. Extra-abdominal desmoid is a rare tumour. On the extensive review of literature, we found only one case of extraabdominal desmoid occurring at digit. We present a case of extraabdominal desmoid-type fibromatosis, which arose in index finger. A 65 year-old woman presented with gradually increasing swelling in the right index finger since one year. Clinical examination showed ulcerative cauliflower-like growth over the right index finger, which made clinician to diagnose it as a case of squamous cell carcinoma, which turned out to be desmoid-type fibromatosis on histopathological examination. Extra-abdominal desmoid tumor is a locally aggressive tumor, despite being histologically benign.We are presenting this case for its rarity.

 

Introduction

The term “desmoid” refers to the hard, tendon-like appearance of the tumor. Nichols in 1923 was the first to recognize and define extra-abdominal desmoid tumor.[1] Desmoid tumours are rare, accounting for 0.03% of all neoplasms and with an estimated incidence rate of 2-4 per million per year with a slight female preponderance and peak incidence in the third and fourth decades.[2] These tumours are more aggressive in younger patients, with recurrence rates up to 87%.[3] Fibromatoses are a group of benign fibrous tissue proliferations characterized by infiltrative growth and a tendency to recur locally without metastasis. The two major groups are superficial (fascial) and deep (musculoaponeurotic). The deep fibromatoses are characterized as extraabdominal, abdominal, and intraabdominal.[4] In various studies, 28–69% of desmoids were intraabdominal (mesenteric or pelvic) or abdominal wall, and the remaining were extraabdominal.[5] Extra-abdominal desmoid fibromatosis arises principally from the connective tissue of muscles and the overlying fascia or aponeurosis (musculoaponeurotic fibromatosis). It chiefly affects the muscles of the shoulder and pelvic girdles and the thigh of adolescents and young adults.

Case Report

A 65 year-old woman presented with a swelling in right index finger since last 6 months. The swelling was associated with dull aching pain. Clinical examination revealed a hard, ulcerated cauliflower lesion measuring 8x7.5x4.0 cm. over the right index finger. (fig 1) The tumour was 1.5 cm away from surgical margin. Bone was not involved by the tumour. (fig 2) Axillary lymph nodes were enlarged. Clinical diagnosis made was squamous cell carcinoma of right index finger with axillary node involvement.

A surgical amputation of the finger was done and sent for histopathological examination. Cut section of mass was whitish homogenous and fleshy. (fig 3) We also received 4 lymph nodes largest measuring 2x1.8x1.5cm, smallest measuring 0.5x0.4cm.

Microscopic examination showed skin with epidermis exhibiting extensive surface ulceration. The dermis showed well-defined tumor mass composed of uniform oval to spindle cells of fibroblast type and spindle cells of myofibroblast type showing variable cellularity with collagenised areas in between.(fig 4) Also seen were few scattered lymphocytes and plasma cells, with focal lymphoid aggregates. Tumor cells showed infiltration between deeper fat and muscle fibres. (fig 5) Masson’s Trichrome showed presence of collagen intervening between tumour cells. (fig 6) Periosteal tissue and bone were free of tumor. Lymphnodes were free of tumour.

Based on these gross and microscopic findings, we gave a diagnosis of extraabdominal desmoid fibromatosis of right index finger.

Figure 1
Figure 1: Index finger with noduloulcerative growth

Figure 2
Figure 2: X ray showing growth with bone free of tumor.

Figure 3
Figure 3: cut surface of the growth-homogenous, greyish white, fleshy

Figure 4
Figure 4: Low power view (H & E stain) showing spindle shaped cells and intervening collagen pointed out by arrow.

Figure 5
Figure 5: Low power view (H & E stain) of the tumour showing infiltration of the fat

Figure 6
Figure 6: Low power view (Masson’s Trichrome stain) showing collagen (pointed out by white arrow)interlaced between tumour cells

Discussion

Extraabdominal desmoid-type fibromatosis (also known as extraabdominal desmoid, well-differentiated nonmetastasizing fibrosarcoma, and aggressive fibromatosis) arises from the connective tissue of muscle and its overlying aponeurosis or fascia.[4]

Masson and Soule believed the term “desmoid tumor” to be acceptable when referring to a specific form of an infiltrative fibrous growth that may appear in various locations, often recurs locally, and does not metastasize, but under certain extenuating circumstances may cause loss of a limb or more rarely death.[6]

Extra-abdominal desmoid tumour is a non-metastasizing fibrous tumour. It is characterised by infiltrative invasion of soft tissues and a high propensity for local recurrence after surgical excision.[4,7]

A case of extra-abdominal fibromatosis, is a rare entity.Although the etiology of extraabdominal desmoid-type fibromatosis is not known, some associations include genetics, hormonal status (related to pregnancy and often regressing after delivery), and prior trauma.[8] The condition most commonly manifests between puberty and age 40 years, with the highest frequency of cases occurring between 25 and 35 years of age.[4] Our patient is a 65 year old female, this age is higher than that reported in literature. Women are more likely to be affected than men and the tumors are more aggressive in younger patients. [9] Patients younger than 30 years have higher relapse rates than do those over 30 years of age.[10] The tumors are also multicentric in approximately 10%–15% of cases.[11]

Extraabdominal desmoid-type fibromatosis often begins as a painless, deep soft-tissue mass in the lower extremity (eg, thigh, foot, or ankle), upper extremity (eg, shoulder or hand), or the head and neck.[12] In our case patient presented with dull aching pain in right index finger.

Grossly extraabdominal desmoid-type fibromatosis are usually composed of nonencapsulated gray-white tissue confined to the musculature and overlying fascia or aponeurosis.[4,13] The tumors are firm and glistening white on cut section and resemble scar tissue.[4] Our case presented as noduloulcerative mass and on cut section, it was whitish homogenous and fleshy.

The tumors are composed of alternating bundles of locally infiltrating, monomorphic elongated, spindle-shaped fibroblast and myofibroblast bundles within a collagenous Stroma.[10,14] Mitotic activity and cellularity are low (four mitoses per 50 high-power field).[15] Necrosis and hemorrhage are absent features in this tumor, we had similar findings in our case .

The cells are usually actin-positive and CD34- and S100-negative. [15] Masson’s Trichrome stain and reticulin stain can be used to reveal collagen interlaced between tumor cells.[4] In our case, Masson’s Trichrome stain showed collagen between tumour cells. Extraabdominal desmoids are intimately associated with muscle and fascia, with the most common locations being the shoulder and upper extremity (33%) gluteal region and lower extremity (30%) chest wall or back(17%), and head and neck (10%) .[2, 16, 17] Isolated cases of breast desmoids have been reported.[18]

In our case tumor is seen involving right index finger which is extremely rare site. In a case study done by Easter and Halasz with a nineteen cases of desmoids tumours, twelve cases were of extraabdominal desmoids, out of which single case of digital fibromatosis with recurrence had been mentioned.[19]

To the best of our knowledge, ours is the second case of extra abdominal desmoid tumor occurring at the digital site.

Surgery and radiation therapy, either alone or in combination, have been used to treat extra-abdominal desmoids. For potentially resectable lesions, surgery provides excellent local control, even in those with recurrent disease.[20] In our case, surgical amputation was done and clinical follow up was advised.

Conclusion

A case of extra-abdominal fibromatosis in a female occurring at index finger is an extremely rare entity. To the best of our knowledge, ours is the second case of extra-abdominal desmoid tumor occurring at the digital site, which clinically mimicked squamous cell carcinoma. Our patient is a sixty three year-old female, an age is greater than that reported in literature. Desmoid tumors are a challenging clinical condition with locally aggressive behaviour and a strong tendency for recurrence. Histopathological confirmation is a must for diagnosis. Management options include observation, surgical resection, radiotherapy, conventional chemotherapy, hormonal agents, and newer molecular targeted agents. A multidisciplinary approach tailored to the individual patient is usually needed, depending on the location, local effects, and clinical course.

Acknowledgements

We are extremely thankful to department of Surgery for providing clinical details, photographs and X-ray picture of the lesion

References

1. Das Gupta TK, Brasfield RD, O’Hara J. Extra-abdominal desmoids: A clinicopathological study. Ann Surg. 1969 Jul;170(1):109-21
2. Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol 2001;27:701–706
3. Romero JA, Kim EE, Kim CG, Chung WK, Isiklar I. Different biologic features of desmoid tumors in adult and juvenile patients: MR demonstration. J Comput Assist Tomogr 1995; 19:782–787
4. Weiss SW, Goldblum JR, eds. Enzinger and Weiss’s soft tissue tumors. 4th ed. St Louis, Mo: Mosby, 2001; 320–329.
5. de Camargo VP, Keohan ML, D’Adamo DR, et al. Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer 2010; 116:2258–2265.
6. Masson JK, Soule EH. Desmoid tumors of the head and neck. Am J Surg. 1966 Oct;112(4):615-22.
7. Pignatti G, Barbanti-Bròdano G, Ferrari D, et al. Extraabdominal desmoid tumor. A study of 83 cases. Clin Orthop Relat Res 2000; 375:207-13.
8. The enigma of desmoid tumors. Curr Treat Options Oncol 2006; 7(6): 438–443.
9. Woertler K. Soft tissue masses in the foot and ankle: characteristics on MR imaging. Semin Musculoskelet Radiol2005; 9(3): 227–242.
10. Ballo MT, Zagars GK, Pollack A, Pisters PW, Pollack RA. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol 1999; 17(1): 158–167.
11. Robbin MR, Murphey MD, Temple HT, Kransdorf MJ, Choi JJ. Imaging of musculoskeletal fibromatosis. RadioGraphics 2001; 21(3): 585–600.
12. Kransdorf MJ. Benign soft-tissue tumours in a large referral population: distribution of specific diagnoses by age, sex, and location. AJR Am J Roentgenol1995; 164(2): 395–402.
13. Pakos EE, Tsekeris PG, Goussia AC. Desmoid tumours of the extremities and trunk: a review of the literature. Int Orthop2005; 29(4): 210–213.
14. Wcislo G, Szarlej-Wcislo K, Szczylik C. Control of aggressive fibromatosis by treatment with imatinib mesylate: a case report and review of the literature. J Cancer Res Clin Oncol2007; 133(8): 533–538.
15. Ferenc T, Sygut J, Kopczynski J, et al. Aggressive fibromatosis (desmoid tumors): Definition, occurence, pathology, diagnostic problems, clinical behaviour, genetic background. Pol J Pathol2006; 57(1): 5–15.
16. Dinauer PA, Brixey CJ, Moncur JT, Fanburg- Smith JC, Murphey MD. Pathologic and MR imaging features of benign fibrous soft-tissue tumors in adults. Radio Graphics 2007; 27:173–187
17. Murphey MD, Ruble CM, Tyszko SM, Zbojniewicz AM, Potter BK, Miettinen M. From the archives of the AFIP: musculoskeletal fibromatoses—radiologic-pathologic correlation. Radio Graphics 2009;29:2143–2173
18. Okamoto K, Kurihara Y, Imamura K, et al. Desmoid tumor of the breast: the role of proton magnetic resonance spectroscopy for a benign breast lesion mimicking a malignancy. Breast J 2008; 14:376–378
19. David W. Easter, Nicholas A. Halasz. Recent trends in the management of desmoid tumors. Ann Surg1989;210(6): 765-768.
20. Buitendijk S, Ven CP, Dumans TG, Hollander JC, Nowak PJ, Tissing WJ, et al. Paediatric aggressive fibromatosis: A retrospective analysis of 13 patients and review of literature. Cancer. 2005;104(5):1090–1099. doi: 10.1002/cncr.21275.

Author Information

Atul Jain
PG student, Department of Pathology, Mahatma Gandhi Medical College and Research Institute

Dhananjay Shrikant Kotasthane
Professor and HOD, Department of Pathology, Mahatma Gandhi Medical College and Research Institute

G Koteeswaran
Associate Professor, Mahatma Gandhi Medical College and Research Institute

Download PDF

Your free access to ISPUB is funded by the following advertisements:

 

 

BACK TO TOP
  • Facebook
  • Google Plus

© 2013 Internet Scientific Publications, LLC. All rights reserved.    UBM Medica Network Privacy Policy

Close

Enter the site

Login

Password

Remember me

Forgot password?

Login

SIGN IN AS A USER

Use your account on the social network Facebook, to create a profile on BusinessPress