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Year : 2014 | Volume : 2 | Issue : 1 | Page : 55 - 56  


Case Reports
An Unusual case of large Desmoid tumour: A case report

Kishori D1, Sudhir Kumar V2, Indira V3             

1Assistant Prof, 2Associate Prof, 3Prof and HOD, Department of Pathology, Malla Reddy Institute of Medical Sciences, Suraram, Hyderabad.

Abstract:

Desmoid are rare soft tissue tumours most commonly seen in females between 20-40 years of age.They are known for their infiltrative pattern of growth and local recurrence. Diagnosis through clinical presentation imaging and histopathology forms the key to differentiate Desmoid tumours from other soft tissue sarcomas like Fibro sarcomas to offer correct treatment.Here we report one such case of Desmoid tumour of the anterior abdominal wall in a female patient which was removed by radical resection.

Key words: Desmoid tumour, abdominal wall, local recurrence, spindle shaped cells.

Corresponding Author: Dr. Kishori D, Department of Pathology, Malla Reddy Institute of Medical Sciences, Hyderabad Email: 21.kishori@gmail.com

Introduction:

Desmoid tumors are rare locally invasive neoplasms constituting 3% of the entire soft tissue tumours. They are commonly seen in female patients in their reproductive age group. They have a tendency for local recurrence, thus having an intermediate malignant behavior. The most common site is anterior abdominal wall, though other rare sites like deep intra-abdominal, back etc. have been described. [1]

Here we report one such case of Desmoid tumor in a 30 year old pregnant patient with no previous history of trauma or surgery.

Case report:

A 30 year old female patient presented to the surgical out-patient department with a mass on the anterior abdominal wall with fourteen weeks of gestation.

Clinically the swelling was located in the hypo gastric region extending in to both the lumbar regions, measuring 20x18cm in size, firm, non-tender and mobile only horizontally. The patient underwent a medical termination of pregnancy and returned within two months. The patient stated that the mass was progressively increasing in size. There was no other relevant history or previous history of trauma or surgery. All the basic blood parameters were within normal limits. Ultrasound imaging revealed a well defined isoechoiec lesion in the anterior abdominal wall in the para umbilical region suggesting a Desmoid tumor.

Radical resection of the tumour along with the infiltrating Rectus muscle was done and the anterior abdominal wall was reconstructed using a polypropylene mesh. The resected tumour was sent for histopathology.

At the department of Pathology, MRIMS we received a grey brown to dark brown large soft tissue mass weighing 1.2Kg and measuring 21 X 17 X 7cmwith attached muscle tissue. Cut-section showed homogenous fleshy grey white tumor with irregular edges infiltrating adjacent tissue (fig 1). The tissue was grossed and routinely processed and stained with hematoxylin & Eosin. Microscopically the tumor was composed of spindle shaped cells arranged in bundles, fascicles and occasional storiform pattern. There are foci showing entrapped muscle bundles, giant cell like cells and normal neural elements (fig 2). Individual tumour cells show indistinct cell borders with moderate amount of pale eosinophilic cytoplasm with a spindle shaped mildly pleomorphic nuclei with vesicular chromatin and inconspicuous nucleoli, embedded in a collagenous matrix (fig 3). Mitotic activity was rarely noted (0-1/HPF). A diagnosis of Desmoid tumor was offered on histomorphology. The patient was discharged on 7th post operative day following an uneventful post-operative period.

Discussion:

Desmoid tumour is a rare aggressive fibromatosis occurring predominantly in females in the fertility age group. Though locally infiltrating, they never metastasise. Based on the location they can be abdominal, intra-abdominal or extra-abdominal, but don’t vary in morphology. Abdominal desmoids arise from musculo-apo-neurotic structures of the anterior abdominal wall viz., Rectus muscle, internal oblique muscle and their fascia.Other rare locations like fascia of tendons and ligaments of arm and legs and occasionally head and neck region have also been described. There are few cases of retroperitoneal Desmoids reported, commonly associated with familial adenomatous polyposis (FAP). [2] Grossly, the tumour is irregular with infiltration and is gritty to cut owing to the collagen content.Microscopically, the tumour tissue is composed of spindle shaped cells with indistinct cell borders with pale eosinophilic cytoplasm with spindle shaped nuclei with vesicular nuclei and small one or two nucleoli.Mitosis is conspicuous by its absence. There is characteristic entrapment of muscle bundles which at places resemble giant cells (giant cell like cells). They can be differentiated from Fibro sarcomas by lack of marked pleomorphism and high mitotic activity.   Though, the tumor is benign it is known for its local recurrence, which occur at a frequency of 40-45% if not properly resected. [1, 3]

Though the cause of the tumor is not known some believe it to be a reactive process and others neoplastic one. Female preponderance could be attributed to hormonal effect of oestrogen and tumours are known to regress following menopause. [4] Desmoid tumours are commonly associated with previous abdominal or pelvic surgery [5] or trauma, familial adenomatous polyposis (FAP) and with Gardener’s syndrome. Recent advances suggest mutations in either APC gene or β-catenin gene to be present in almost all the cases. [6]

Our patient presented with a painless swelling measuring 21 X 18cm with 14 weeks of gestation. After undergoing amedical termination of pregnancy, the patient was posted for radical resection. Intra - operatively the tumour was found to be diffusely infiltrating the rectus.It was resected along with the Rectus muscle and sent for histopathology. Though, sonologically it is seen to be a well circumscribed lesion, grossly it was an irregular tumour infiltrating surrounding tissue. Histomorphology revealed features of Desmoidtumour with spindle shaped cells arranged in fascicles and bundles with vesicular nuclei and small nucleoli in a collagenous network and entrapped muscle bundles. There is infrequent mitosis and with peripheral normal neural elements. These tumours are described to evolve over time with the deposition of more collagen. Initially they are more cellular with sparse areas of collagenisation. In the next stage there are areas with central and peripheral hyalinization followed by a later stage of increased fibrous deposition and less cellularity. [7]

A complete resection of the tumour along with infiltrating muscles with reconstruction was done in this case.Though the patient had uneventful post op period, regular follow up is needed to detect any early recurrences which are seen usually following an incomplete section. Local recurrences present during the first six months post operatively and never known to metastasise. [8] The histopathological examination is the key to rule out any Fibrosarcomas as the treatment modality differs. Fibrosarcomas are more cellular with characteristic storiform arrangement of cells. The cells are markedly pleomorphic with high mitotic activity and areas of necrosis may be noted. [9]

Conclusion:

The classical clinical presentation like female gender, anterior abdominal wall location along with imaging and microscopy confirm a diagnosis of Desmoid tumour even though it is a rare neoplasm.Complete radical resection with regular follow up help yielding a good prognosis to the patients.

References:

  1. Economou A, Pitta X, Andreadis E, Papapavlou L, Chrissidis T: Desmoid tumor of the abdominal wall: a case report. J Med Case Rep. 2011;25:5:326.
  2. Lynch HT, Fitzgibbons R Jr: Surgery, desmoids tumors, and familial adenomatous polyposis: case report and literature, review Am J Gastroenterol 1996, 91(12):2598-601.
  3. Overhaus M, Decker P, Fischer HP, TextorHJ, Hirner A: Desmoidtumors of the abdominal wall: a case report. World J Surg Oncol 2003,1(1):11.
  4. de Bree E, Dimitriadis E, Giannikaki E, Chryssou EG, Melissas J: A giant pregnancy-associated intraabdominaldesmoid tumour: not necessarily a contraindication for subsequent pregnancy. World J Surg Oncol. 2013;11:277.
  5. De Cian F, Delay E, Rudigoz RC, Ranchere D, Rivoire M. Desmoid tumor arising in a cesarean section scar during pregnancy: monitoring and management Gynecol Oncol 1999,75(1):145-148.
  6. George T. Calvert, Michael J. Monument, Randall W. Burt, Kevin B. Jones, and R. Lor Randall, “Extra-Abdominal DesmoidTumors Associated with Familial Adenomatous Polyposis,” Sarcoma, vol. 2012, Article ID 726537, 11 pages, 2012. doi:10.1155/2012/726537.
  7. Vandevenne JE, De Schepper AM, De Beuckeleer L, Van Marck E, Aparisi F, Bloem JL et al. New concepts in understanding evolution of desmoidtumors: MR imaging of 30 lesions. Eur Radiol 1997;7(7):1013-9.
  8. Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan MF. The enigma   of desmoidtumors. Ann Surg. 1999;229(6):866-72.
  9. Kumar V, Khanna S, Khanna AK, Khanna R. Desmoid tumors: experience of 32 cases and review of the literature. Indian J Cancer. 2009;46(1):34-9.