Pediatric Fibroblastic and Myofibroblastic Lesions

Abstract

Fibrous lesions of infancy and childhood are a heterogeneous group of entities composed predominantly of fibroblasts and myofibroblasts, ranging from reactive lesions to neoplasms with a range of malignant potential. Although rare, their correct recognition by histopathology is important clinically as they exhibit a wide range of behaviors and may be associated with distinct underlying syndromes. Contributions from molecular diagnostics have enabled more accurate diagnosis, and have changed our concepts of some tumor types. In this review, we discuss the clinicopathologic spectrum of fibroblastic and myofibroblastic lesions of childhood and adolescence.

Soft Tissue Special Issue: Perivascular and Vascular Tumors of the Head and Neck

“…Nuclear expression of beta-catenin is a result of mutations in the corresponding CTNNB or ABC gene, leading to reduced degradation of the protein. Androgen receptor expression is also typical for this lesion [11,12,14]. Benign vascular lesions as vascular malformations could be a differential diagnosis.…”

“…Nasopharyngeal Angiofibroma (JNA) (Juvenile) nasopharyngeal angiofibroma (JNA) is an uncommon benign fibrovascular lesion originating in the nasopharyngeal region of mainly male adolescents and young adults aged between 14 and 25 years. Women and older patients are rarely affected [11][12][13]. JNA involves the nasopharynx and dorsolateral aspect of the nasal cavity.…”

“…JNA involves the nasopharynx and dorsolateral aspect of the nasal cavity. Although morphologically benign it can cause destructive involvement of the paranasal sinuses, orbit and skull base with intracranial extension [12,13]. Symptoms are commonly obstruction and epistaxis.…”

“…Symptoms are commonly obstruction and epistaxis. Individual cases have been associated with familial adenomatous polyposis [12,14]. Recurrences after surgery are reported in up to 57% of cases [11].…”

Fibromatosis of the Sigmoid Colon With CTNNB1 (β-Catenin) Gene Mutation, Arising at the Site of Ileocolic Anastomosis for Resection of Gastrointestinal Stromal Tumor

“…Aggressive (deep or desmoid-type) fibromatoses are locally infiltrative, collagen-forming neoplasms with the potential for recurrence but not metastasis. 4,5 They can arise at any anatomic site, although there are distinct subsets in the abdominal wall and intra-abdominally. Their biologic behavior is variable; they tend to be slowly enlarging, deep-seated, painless masses but their clinical effects primarily manifest according to their site, and intra-abdominal fibromatoses can present with pain, change in bowel habit, bowel obstruction, ischemia or rectal bleeding.…”