Isolated Infratemporal Fossa Desmoid Fibromatosis: A Rare Case Report and Review of Literature

This article discusses the management of a 2-year-old child with desmoid fibromatosis (DF) of the infratemporal fossa (ITF) along with literature review.

Mesenteric fibromatosis (MF) is a rare, locally aggressive tumor without distant metastasis, which has a high recurrence rate. Based on its location, it is classified as intra-abdominal, from abdominal wall, and extra-abdominal. The incidence of cystic-solid, retroperitoneal tumors is very low in comparison to other MF forms. Intra-abdominal MFs are asymptomatic in early stages, but their symptoms appear late in the tumor course. There is no specific imaging finding since radiological diagnosis is mostly impossible. Thus, diagnosis is made histopathologically. Nowadays, there is no consensus about its treatment although surgical resection is widely used. In the present study, a very rare case of cystic-solid retroperitoneal MF associated with separate synchronous skin tumors is reported.

Here we report a case of 65-year-old man who came to the surgery department with complaints of pain in abdomen and constipation for 5 days. He had no history of any trauma or surgery. The family history was not significant. On physical examination, a lump of 10 x 6 cm was palpated in lower abdomen. The lump was firm, non-tender with diffuse border. The lower border was not palpable. Relevant blood investigations were done and were in the normal range. X-ray findings showed features suggestive of subacute intestinal obstruction. MRI Findings Revealed a large mass arising from the anterior lower abdominal wall muscles. Surrounding organs appear normal. No enlarged lymph nodes were detected. No ascites. Features were suggestive of desmoid tumour.

A 45-year-old woman presented with pain and gradually progressive lump in abdomen since 6 months in right iliac fossa with no history of trauma. On local examination the mass was immobile, firm in consistency with no local tenderness. She had a surgical scar mark, from her two caesarean sections. No other significant surgical history was present. In view of above findings she was advised CT scan. On CT there was a well circumscribed heterogeneously enhancing mass lesion with poorly enhancing central area in anterior abdominal wall towards right inguinal region appearing to be arising from right rectus abdominis muscle measuring approximately 6.6 x 6.5 x 4.7 cms in size.

This study compared the clinical and functional outcomes of patients initially treated with observation or medical treatment with those of patients treated with local treatment (surgery alone or surgery with adjuvant radiotherapy) to confirm whether observation or medical treatment is an appropriate first-line management approach for patients with desmoid tumors. We retrospectively reviewed the medical records of 99 patients with histologically confirmed primary desmoid tumors treated between 1978 and 2018. The median follow-up period was 57 months. We evaluated event-free survival, defined as the time interval from the date of initial diagnosis to the date of specific change in treatment strategy or recurrence or the last follow-up. An event (specific change in treatment strategy or recurrence) occurred in 28 patients (28.3%). No significant difference in event-free survival was found between the first-line observation/medical treatment and local treatment groups (p = 0.509). The median Musculoskeletal Tumor Society score of the patients treated with first-line local treatment was 29 (interquartile range [IQR], 23-30), whereas that of the patients managed with first-line observation or medical treatment was 21 (IQR, 19-29.5). First-line observation or medical treatment was more frequently chosen for larger tumors (p = 0.045). In the patients treated with local treatment, local recurrence was not related to the surgical margin (p = 0.976). Upfront surgery is not advantageous compared to more conservative treatments such as observation or medical treatment for patients with desmoid tumors.

In the context of a post-treatment testicular germ cell tumour, an abdominal lesion found on surveillance CT studies led to a differential diagnosis, including recurrent germ cell tumour. We report the case of a 48-year-old man who was noted to have a new interval soft tissue lesion on a surveillance CT scan, 5 years after initial orchidectomy and chemotherapy. Excision of this lesion and histopathological review revealed an intra-abdominal desmoid.