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Desmoid Tumor of the Anterior Abdominal Wall ABSTRACT Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and also formerly termed fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. These benign tumors are associated with a fertile women after pregnancy as in this case who/whereby a felt small mass was felt in a woman’s her right iliac fossa after her last labor//following delivery. increasing in size by time. Many patients have a history of breast cancer or breast surgery as in this case/patient who had fibroadenoma of breast. Many patients usually have a history of breast cancer or breast surgery and in the case of our patient, there was history of fibroadenoma. Preoperative evaluation included hemoglobin%, ultrasound and CT scan. The mass was excised surgically without abdominal reconstruction and subsequent histology revealed desmoid tumor. The patient discharged with uneventful recovery. Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and also formerly termed fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. These benign tumors are associated with fertile women after pregnancy and many patients usually have a history of breast cancer or breast surgery. This is a case report of a woman who gave history of fibroadenoma and who was found to have had a small mass her right iliac fossa following delivery. The mass was found to be within the abdominal wall after abdominal UltraSound (US) and Computed Tomography (CT) scan of the abdomen was done. Full blood count, Urea, electrolytes and creatinine were done and found to be normal. The mass was excised surgically without abdominal reconstruction and subsequent histology revealed desmoid tumor. The patient discharged with uneventful recovery. Keywords: Desmoid tumor, Anterior abdominal wall, Gardner syndrome Introduction Desmoid tumors are (also called desmoids fibromatosis) are rare soft tissue tumors accounting for 0.03% of all tumors [2]. The term desmoids was coined by Muller in 1838 and is derived from the Greek word desmos, which means tendon like [1]. The tumors are slowly growing benign fibrous tumors without any metastatic potential but a have strong tendency to invade locally and to recur. They are frequently seen in patients with familial adenomatous polyposis (FAP) and are also usually seen in women between puberty and 40 years of age [3]. Desmoid Tumors occasionally exhibit rapid growth and are seen in the abdominal wall, intraabdominally and rarely on the extremities [3,4,5] Surgery, although followed by frequent local recurrence, is generally considered the treatment mainstay. Other treatment modalities include radiotherapy, chemotherapy (using cyclophosphamide, doxorubicin, tacarbazin, methotrexate, vincristine and actinomycinD). Hormonal therapy (tamoxifen) with either steroid or a potent nonsteroidal anti-inflammatory drug (e.g., Indomethacin, sulindac or Colchicine) has/have been used both preoperatively and postoperatively [6] CASE REPORT: A 23-year- old married housewife not pregnant presented with a 9 months history of a painless right lower abdominal painless mass/swelling which had developed her last delivery which had been by cesarean section. She was multiparous and was not pregnant at the time of presentation and she gave history of the swelling having progressively increased in size. She reported having experienced dizziness, poor appetite and weight loss but there was no alteration in bowel habits. She had also started felling nauseated one month prior to coming to hospital and also complained of excessive menstrual blood loss. A breast lump (fibroadenoma) had been excised from one of her breasts 2 years prior during her last pregnancy. On examination, she was anxious, thin, looked pale, not jaundiced, and had no palpable lymph nodes. She had a cesarean section scar seen, no abdominal striae and there was visible an obvious large swelling at the right lower abdominal quadrant with no overlying skin changes. The mass was not moving with respiration, was firm, fixed, non-tender, well-defined and it’s dimensions were 15 cm by 15 cm. with soft surface and locally restricted mobility, not tender ,hard mass, clear edge, not disappeared on raising the patient from supine to sitting posture and no palpable intraabdominal organs were noticed. INVESTIGATIONS Full blood count, urea, electrolytes and creatinine were normal. Abdominal US was reported as showing evidence of a well-defined, isogonic solid mass located at the right side of lower abdominal cavity, contiguous with the anterior abdominal wall measuring about 11 cm. by 7.5 cm and containing dots of calcification. The CT scan of abdomen (Fig.1) confirmed an anterior abdominal wall mass (9.8 cm x 8 cm) with a smooth outline and with no evidence of invasion of the surrounding structures. Fig.1 (CT scan of the abdomen) Operation: Under general anesthesia and through a right lower paramedian incision the mass was excised from the anterior abdominal wall. It was located between rectus abdominis muscle and the posterior rectus sheath. Please label figure 2&3 properly with a caption for each Fig.3 (Fig.2,3 Lateral view of the mass attached to posterior rectus sheath.) Fig.4 (View of the posterior rectus sheath after complete removal of the mass.) . Fig.2 PATHOLOGY: Histopathological ( Fig.5&6 ) result was benign spindle cell lesion C/W fibromatosis (Desmoids tumor ) with no evidence of malignancy is seen Fig.5 Fig.6 Fig.5,6 ( Histopathological photos confirming Desmoids tumor) DISCUSSION: Desmoid tumors are rare and they account for about 0.03 percent of all neoplasms and less than 3 percent of all soft tissue tumors [ref]. The estimated incidence in the general population is two to four per million population per year and they are slightly more common in women than in men [7,8]. Most desmoids arise sporadically, although approximately 2 percent are associated with Familial Adenomatous Polyposis (FAP). Desmoid tumors affect between 10 to 20 percent of patients with FAP [9]. They are an unusual cause of a breast mass, and they may be mistaken for a primary or recurrent breast cancer. Many patients have a history of breast cancer or breast surgery. In a series of 32 patients with a breast desmoid, eight (25 percent) had a previous history of breast cancer, and 14 (44 percent) had prior breast surgery [10]. CONCLUSION: REFERENCES 1.Case Reports in Surgery Volume 2013, Article ID 780862, 4 pages http://dx.doi.org/10.1155/2013/780862 Giant Desmoid Tumor of the Anterior Abdominal Wall in a Young Female: A Case Report .Mahim Koshariya, Samir Shukla, Zuber Khan, Vaibhav Vikas,Avinash Pratap Singh, Puspendra Baghel, Varun Pendro, Vishal Kirti Jain,Shrikant Jagdish Jai, Sanjeev Kumar, andM. C. Songra 2. Familial Cancer 1: 111–119, 2001.2002 Kluwer Academic Publishers. Printed in the Netherlands.Desmoid tumour in familial adenomatous polyposis.A review of literature Anne Lyster Knudsen and Steffen Bülow The Danish Polyposis Register, Department of Surgical Gastroenterology, Hvidovre University Hospital,Hvidovre, Denmark 3. Knudsen AL, Bulow S (2001) Desmoid tumour in familial adenomatous polyposis. A review of literature. Fam Cancer 1:111– 119 4.Reitamo JJ, Scheinin TM, Hayry P (1986) The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 151:230–237 5. Rodriguez-Bigas MA, Mahoney MC, Karakousis CP, Petrelli NJ (1994) Desmoid tumors in patients with familial adenomatous polyposis. Cancer 74:1270–1274 6. Gwynne-Jones DP, Theis JC, JeVery AK, Hung NA (2005) Longterm follow-up of a recurrent multifocal desmoid tumour treated with tamoxifen: a case report. J Orthop Surg (Hong Kong) 13:174– 177 7. Bertario L, Russo A, Sala P, et al. Genotype and phenotype factors as determinants of desmoid tumors in patients with familial adenomatous polyposis. Int J Cancer 2001; 95:102. 8.Mankin HJ, Hornicek FJ, Springfield DS. Extra-abdominal desmoid tumors: a report of 234 cases. J Surg Oncol 2010; 102:380. 9. Järvinen HJ. Desmoid disease as a part of familial adenomatous polyposis coli. Acta Chir Scand 1987; 153:379. 10.Neuman HB, Brogi E, Ebrahim A, et al. Desmoid tumors (fibromatoses) of the breast: a 25-year experience. Ann Surg Oncol 2008; 15:274.