A giant pregnancy-associated intra-abdominal desmoid tumour: not necessarily a contraindication for subsequent pregnancy
© de Bree et al.; licensee BioMed Central Ltd. 2013
Received: 24 June 2013
Accepted: 19 September 2013
Published: 16 October 2013
Desmoid tumours are rare mesenchymal tumours, often locally invasive and characteristically associated with a high local recurrence rate after resection. A potential aetiological role for female hormones is indicated. Pregnancy-associated desmoid tumours are almost exclusively located in the abdominal wall. An essential issue is how to counsel women who have had a pregnancy-associated desmoid tumour and subsequently wish to bear a child. A considerably rare case of a patient with a resection of a giant pregnancy-associated, 33 cm in diameter, intra-abdominal desmoid tumour is presented. After a subsequent pregnancy, the patient delivered healthy twins 26 months later. Fifty-four months after treatment, there are no signs of recurrent or second desmoid tumour. Although rarely located in the abdomen, pregnancy-associated desmoid tumours should be included in the differential diagnosis of intra-abdominal tumours detected during or shortly after pregnancy. Based on this case and a few others reported in the literature, subsequent pregnancy does not necessarily seem to be a risk factor for recurrent or new disease.
KeywordsAggressive fibromatosis Desmoid tumour Intra-abdominal tumour Pregnancy
Desmoid tumour, also called aggressive or desmoid-type fibromatosis, is a rare monoclonal, fibroblastic proliferation. Although histologically benign and unable to give rise to metastases, desmoids are often locally invasive and characteristically associated with a high local recurrence rate after resection. While this fibroblastic disorder may be observed in nearly every part of the body, desmoids occur most commonly in extremities. Intra-abdominal location is observed only in a small proportion of patients .
Aetiology of desmoid tumours is incompletely defined. Numerous acknowledged factors are associated with their development . Increased incidence occurring during and after pregnancy, as well as following use of oral contraceptives, the preponderance of women of reproductive age in many series, anecdotal reports of spontaneous tumour regression during menopause, expression of oestrogen beta receptor and reports of tumour regression with anti-oestrogen treatment are all factors which indicate a potential aetiological role for female sex hormones [1, 2]. Pregnancy-associated desmoids are almost exclusively located in the abdominal wall [2, 3]. Herein we report on a young woman with a giant intra-abdominal desmoid tumour diagnosed 3 weeks after giving birth and resected soon afterwards. The location of a pregnancy-associated desmoid tumour in the abdomen is unusual. Further, due to hormonal influences, a subsequent pregnancy may theoretically be a risk factor for a second desmoid or recurrent disease. Neither during nor after the subsequent pregnancy was recurrence or development of a second desmoid tumour observed in our case. The literature on this topic is herein reviewed.
The patient did not receive any adjuvant treatment. She became spontaneously pregnant, despite the recommendation that she postpone pregnancy to allow for a 2-year disease-free follow-up period. Nevertheless, she delivered healthy twins by Caesarean section 26 months after initial treatment for the desmoid tumour. Fifty-four months after resection of the desmoid, she is in excellent condition without any sign of tumour recurrence on abdominal sonography and MRI, or of development of a second desmoid tumour.
The enormous tumour size in the present case is noteworthy. Primary desmoids >30 cm in size have only incidentally been reported [4–6]. It remains remarkable that the tumour was not observed at routine repetitive sonography during pregnancy. The optimal treatment of desmoids remains difficult to be determined, due to the rarity, the heterogeneity and the very unpredictable natural history of the disease. Therefore, an individualized approach is warranted . Surgical resection has most commonly been used, while radiotherapy, medical treatment and watchful waiting are alternative options .
Reports of pregnancy-associated desmoid tumours located outside the abdominal wall
Patient’s age (years)
Site of origin
Time of diagnosis
Treatment and outcome
17 weeks of gestation
Excision on the 12th postpartum day; free of recurrence 1 year later
5-6 weeks of gestation
Incomplete excision; excision of recurrence after 2 months; second recurrence after several months, treated by excision and radiotherapy
20 weeks of gestation
Incomplete excision; recurrence after 8 weeks; spontaneous complete regression 9 weeks after delivery
10 days after c.s.
Excision + hormonal therapy, recurrence after 12 months, excision of recurrence + radiotherapy, rapidly second recurrence, complete response for 27+ months with hormonal treatment
23 weeks of gestation
At 23 weeks gestation complete resection, outcome not reported
2nd month of gestation
One month after complete excision no recurrence
Immediately after c.s.
One year after complete excision no recurrence
One year after complete excision no recurrence
2 months postpartum
Radical excision along with small bowel segment, outcome not reported
3 weeks postpartum
Fifty-four months after resection no recurrence
Reports of patients diagnosed with pregnancy-associated desmoid tumour and subsequent pregnancy
Patient’s age (years)
Site of origin
Diagnosis and treatment
Subsequent pregnancy and outcome
Shortly after birth of fourth child, observation
Almost complete regression in size during and after subsequent pregnancy
2, 7 and 4
Recurrence after prior excision, wide resection of the area with 3 local recurrences
Subsequent pregnancy >2 years after resection. No new recurrence during and shortly after pregnancy
Immediately after birth of second child, wide resection
Miscarriage, delivery of healthy child and abortion respectively 15, 24 and 39 months after resection. No recurrence 60 months after resection
Twelve months after pregnancy, wide resection
Subsequent pregnancy 13 months after tumour excision. No recurrence 46 months after resection
After first pregnancy, observation
Increase in diameter from 6 to 10 cm during second pregnancy, excision of the tumour after transvaginal delivery. No data of further follow-up
Three weeks postpartum, resection
Delivery of twins 26 months after resection. No recurrence 54 months after resection
Although pregnancy-associated desmoid tumours are almost exclusively located in the abdominal wall, desmoid tumour should be included in the differential diagnosis of intra-abdominal tumours detected during or shortly after pregnancy. From the sparse literature data and from the case herein presented it seems that subsequent pregnancy is not necessarily a risk factor for recurrent or new desmoid tumour. Based on the median time to eventual desmoid recurrence after resection, it may be advisable for the patient to allow for a 2-year follow-up period before planning an eventual subsequent pregnancy.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We would like to thank Mrs. Eugenia Bolbasis for linguistically reviewing the manuscript.
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