Desmoid Fibromatosis of the Lower Abdominal Wall in Irrua Nigeria

Desmoid fibromatosis (desmoid tumors) is rare tumors. It can occur as intra-abdominal, extraabdominal, or abdominal wall tumor depending on the site. The abdominal wall type is usually sporadic, but few have been associated with familial adenomatous polyposis. They are commonly seen in young females who are pregnant with a history of the previous cesarean section scar or within the 1st year of the last childbirth. There is an association between this tumor, presence of estrogen receptors, and abdominal trauma. We present a 29-year-old Nigerian woman with fungating lower abdominal wall tumor.

In our report, we describe a case of a 13-year old patient with a bifocal intra-abdominal DT, treated unsuccessfully with tamoxifene, and we discuss briefly the existing literature data.

We describe a rare case of aggressive fibromatosis of the larynx occurring in a 72-year-old man. Although several laryngoscopy-driven biopsies were negative for malignancy, total laryngectomy was done since the lesion was not deemed amenable to conservative therapy. No further therapy was administered. Based on the available literature, our data confirm that aggressive laryngeal fibromatosis in adult patients is a locally infiltrating disease. Total laryngectomy with clear margins is needed as to avoid the high risk of local recurrence.

At our institution, we recently observed promising results with pazopanib therapy for DT/AF in adolescent and young adult (AYA) patients. Pazopanib provides a promising, well-tolerated therapy for DT/AF in the AYA population and warrants further study.

Patients with rare tumors have difficulty finding reliable information about their disease. Facebook patient support groups allow patients to educate one another. To investigate how these patients perceive the value of pathologists, both in Facebook groups and real-world patient care. Survey links were posted in 12 Facebook patient groups: 6 with an active pathologist member (angiosarcoma, epithelioid hemangioendothelioma, epithelioid sarcoma, dermatofibrosarcoma protuberans [_2], and desmoid fibromatosis), and 6 without “active” pathologist involvement (aggressive angiomyxoma, chondrosarcoma, Ewing sarcoma, leiomyosarcoma, liposarcoma, and osteosarcoma). Pathologist involvement in Facebook patient groups appears to positively influence patient perception of the importance of pathologists. We hope thesedata will encourage more pathologists to participate in Facebook patient support groups.

Desmoid tumor of the breast is a rare benign entity that usually is mistaken for carcinoma clinically and radiologically. We report two cases of desmoid tumor of the breast detected by mammography screening using digital breast tomosynthesis (DBT). The larger tumor was detected at both full-field digital mammography (FFDM) and DBT. The smaller desmoid tumor, however, was identified only at tomosynthesis. Mammographic and ultrasonographic findings at diagnostic work-up were consistent with carcinoma of the breast. Preoperative needle biopsies could not conclusively diagnose the lesions. Both patients underwent excisional biopsy and histopathology revealed fibromatosis of the desmoid type.