|Year : 2015 | Volume
| Issue : 3 | Page : 185-187
Desmoid tumor in pregnancy: A case report
Laitonjam Sushila Devi1, Annapurna Prabhakara2, S Opendra Singh3, Laiphrakpam Ranjit Singh2
1 Department of Pathology, Regional Institute of Medical Sciences (RIMS), Imphal, Manipur, India
2 Department Obstetrics and Gynaecology, Regional Institute of Medical Sciences (RIMS), Imphal, Manipur, India
3 Department General Surgery, Regional Institute of Medical Sciences (RIMS), Imphal, Manipur, India
|Date of Web Publication||1-Dec-2015|
#512/4. 4th Block, Kushalnagar, Coorg - 571 234, Karnataka
Source of Support: None, Conflict of Interest: None
Desmoid tumors (DTs) constitute a rare fibroblastic proliferative disease. In a female patient presenting a tumor of the lower abdominal wall especially after cesarean section, an endometriotic tumor as well as an aggressive DT should be considered. Here we report a case of pregnancy associated with an anterior abdominal wall DT in a second gravida with previous cesarean, being the first such case from the northeastern region. DTs are histologically benign neoplasms arising from the connective tissue sheath of striated muscles but may exhibit locally aggressive growth patterns and a high rate of recurrence. Large percentages of DTs in females arise in and around pregnancy, perhaps related to trauma from abdominal stretching during fetal growth and due to estrogen levels. It is not clear from currently available data whether pregnancy-associated desmoids are molecularly distinct from other desmoids. Currently, surgery is the most favored treatment for DTs.
Keywords: Desmoid, pregnancy, surgery
|How to cite this article:|
Devi LS, Prabhakara A, Singh S O, Singh LR. Desmoid tumor in pregnancy: A case report. J Med Soc 2015;29:185-7
| Introduction|| |
A desmoid tumor (DT), also known as aggressive fibromatosis, constitutes a rare fibroblastic proliferative disease. As suggested by the name ("desmoid" from the Greek word "δεσμoς" meaning "band-like"), a DT may occur in any musculoaponeurotic or fascial tissue. 
Morphologically, DTs are nonencapsulated, grayish-white lesions consisting of collagenous tissue.  Histologically the lesion is composed of long sweeping and intersecting spindle cells, with varying amounts of collagen deposition in the tumor matrix with low to moderate cellularity and insignificant mitotic activity. 
DTs have diverse clinical behavior, and the prognosis depends on the anatomic location and its relation to vital organs.  The reason(s) for the association between pregnancy and development of DTs remain(s) unclear. Stretching or tearing of the anterior abdominal wall musculature during pregnancy due to fetal growth, hormonal influence (such as estrogen), and immune system changes are theoretical possible explanations for development of a DT.  This case report discusses a rare occurrence of abdominal wall DT during pregnancy.
| Case Report|| |
A 35-year-old second gravida with previous cesarean presented with a lump in the lower abdominal wall in the left lumbar region at 12 weeks of gestation, which was nontender and firm, measuring 3 × 4 cm 2 . The lump gradually increased in size and an ultrasound taken at 24 weeks of gestation revealed a well-defined hypoechoic lesion measuring 8.07 × 3.75 cm 2 in the abdominal wall, left flank [Figure 1]. Aspiration cytology of the lump showed a few benign spindle cells with occasional multinucleated myofibroblasts suggestive of fibromatosis. Ultrasound taken at term pregnancy revealed the same space-occupying lesion (SOL), measuring 9.56 × 5.6 cm 2 [Figure 2]. The patient had an elective cesarean section at 39 weeks of gestation.
|Figure 1: Transabdominal sonography (TAS) showing gravid uterus with a hypoechoic SOL in the left lower abdominal wall at 6 months of gestation|
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At 1 month postpartum, a repeat abdominal ultrasound was taken, which showed a SOL of inhomogeneous echo pattern in the left lumbar region measuring 8.86 × 10 cm, suggestive of DT, [Figure 3]. Magnetic resonance imaging (MRI) of whole abdomen in the following month showed irregular, high-intensity subcutaneous nodules in the anterior abdominal wall in both T1- and T2-weighted images, suggesting fibrolipoma [Figure 4]a and b. The patient underwent wide local excision of the tumor, which weighed 356 g. Under general anesthesia at 6 months postpartum the histopathological examination of the excised tissue showed spindle cell neoplasm of storiform pattern with collagen.
|Figure 4: (a) MRI picture showing high-intensity subcutaneous nodules in T1- and T2-weighted images in left flank (b) MRI picture, coronal view|
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| Discussion|| |
DTs constitute 0.03% of all biopsy-analyzed neoplasms. The female/male ratio ranges 1.4-1.8. DTs are nonencapsulated, grayish-white lesions consisting of collagenous fibroblasts and fibrocytes; they are firm and fixed to the surrounding tissue. It is uncommon to note lymphadenopathy, overlying skin changes, erythema, or dilated veins. , The distinction between a desmoid and a fibrosarcoma is particularly important, as the latter has the capacity for distant metastasis. 
The behaviors of DTs, including growth rates, age predilection, and recurrence rates, often vary with the location of the tumor. The commonest site of presentation in the case of abdominal wall desmoids seems to be the infraumbilical rectus sheath.  DTs are usually sporadic but may be associated with familial adenomatous polyposis syndrome. Approximately 10-15% of patients with polyposis present intra- or extraabdominal DTs. The supposed risk factors of DTs are previous surgical interventions, pregnancy, and hormonal treatment with estrogen. ,
The reasons for the association between pregnancy and the development of desmoids remain unclear. A number of possible explanation have been put forward, none very satisfactory. Desmoids might result from tearing and stretching of the abdominal wall musculature during fetal growth. It is well known that DTs may be associated with trauma of different sorts. Against such a theory stands the fact that in other case reports of pregnancy, an associated tumor has developed in aremote location such as the larynx.  Others have attempted to link the hormonal and immune system changes that occur with pregnancy with desmoid development. DTs often develop during or after pregnancy, or following uptake of oral contraceptive pills. Spontaneous regression is reported to occur after menopause, oophorectomy, and after antiestrogen therapy.  Krentel et al. described a symptomatic DT in a 25-year-old woman with 38 weeks of gestation, in the lower abdominal wall.  Viriyaroj et al. found an aggressive fibromatosis of almost 5 kg in the lower abdominal wall of a 17-year-old pregnant woman.  Shashi et al. reported a 22-year-old woman presenting with an abdominal DT at the fifth month of gestation. 
The recommended treatment is wide surgical excision, if possible, but unresectable tumors may be treated with radiotherapy, anticancer drugs, nonsteroidal anti-inflammatory drugs (NSAIDS) or antiestrogenic compounds. The recurrence rate is high and seems to be related to the achievement of resection margins free of tumor. DTs can become very large if untreated, but they are largely asymptomatic unless they begin to encroach on or invade adjacent organs such as muscles, tendons, nerves, blood vessels, and organs.
| Conclusion|| |
In conclusion, the authors report a rare case of pregnancy associated with DT, the first of its kind in the northeastern region of India. The patient had an uneventful pregnancy followed by cesarean section at term. The patient underwent successful treatment with complete surgical excision of the tumor after 6 months of cesarean section without any postoperative morbidity.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]