Sporadic intra-abdominal desmoid tumor: a unusual presentation.

A case of an abdominal desmoid tumor with vascular mesenteric invasion in a 32-year-old female, over 2 years after pregnancy is presented. Recent development is in favor of a non-aggressive treatment such as ‘wait and see’ procedures. Without radical treatment, these tumors could generate bowel compression or perforation. Due to their location and high risk of complication, surgery is the most fitted option.

 Pharmacologic therapies represent a noninvasive alternative to surgical resection. Pain symptoms require therapeutic regimens that must be modified as the tumor evolves in expression. The individualized pain treatment program utilized may often reflect principles used in both nonmalignant and malignant pain management models. This review seeks to increase awareness of desmoid tumors through a review of the literature and discussion of its pharmacotherapeutic management.

The management of DF has evolved considerably in the last decade from aggressive first-line surgery and radiation therapy to systemic treatment (chemotherapy, hormonal therapy, and targeted therapy) and symptomatic local control (surgery and radiation therapy). Imaging plays an important role in each of these treatment settings. In surgical candidates, computed tomography (CT) and magnetic resonance (MR) imaging are the modalities of choice for assessing resectability and surgical planning. For evaluating recurrence, MR imaging is the modality of choice for extra-abdominal recurrence, whereas CT is the preferred modality for intra-abdominal recurrence. Signal intensity changes at MR imaging can be used to monitor the biologic behavior of certain DFs chosen for expectant management. Response to systemic treatment with anti-inflammatory agents, hormonal therapy (eg, tamoxifen), cytotoxic chemotherapy (eg, doxorubicin, vinblastine, methotrexate), and targeted therapy (eg, sorafenib), as well as to radiation therapy, can be assessed at CT by monitoring size and attenuation changes or at MR imaging by monitoring size, T2 signal intensity, and degree of enhancement. Several patterns of response can be seen at imaging. Imaging also helps in detecting complications associated with systemic therapy and radiation therapy.

To evaluate the rate of local recurrence of extra-abdominal desmoid tumor and compare the outcomes of surgical treatment and conservative treatment. Twenty one patients (14 women and seven men), mean age 33.0±8.7 years old, with a diagnosis of desmoid tumor were evaluated. Recurrence occurred in seven patients (33%) and six of them relapsed within the first 18 months. No significant difference was observed between conservative and surgical treatment. However, a significant difference was observed among patients undergoing wide resection and who experienced improved local control.

Desmoid-type fibromatosis rarely occurs in the oral tongue. Diagnostic imaging, including ultrasound, is useful for delineating the extent of the lesion and can aid in treatment planning. However, definitive diagnosis is made based on the characteristic histologic features, which include cytologically bland spindle cells with surrounding collagenous matrix and minimal mitotic activity.

We present a case of a 14-month-old male with an 8-cm desmoidtumor in the right parapharyngeal space and provide an overview of diagnosis and management of pediatric head and neck fibromatosis. This is the largest desmoid tumor of the parapharyngeal space in the youngest patient described in the literature.