Calcifying Aponeurotic Fibroma in Children: Case Report

Case Report

J Pediatri Endocrinol. 2021; 6(1): 1039.

Calcifying Aponeurotic Fibroma in Children: Case Report

Kessab A1,3*, Chalouah B2,3, Azzakhmam M1,3, Benchakroun M2,3, Bouaabid AS2,3 and Rouas L1,3

1Department of Anatomy Pathological, Laboratory Children’s Hospital Ibn Sina CHU, Morocco

2Department of Orthopedic Surgery, Military Hospital, Morocco

3Faculty of Medicine and Pharmacy of Rabat (FMPR), Morocco

*Corresponding author: Amine Kessab, Pathological Anatomy Laboratory Children’s Hospital Ibn Sina CHU, Faculty of Medicine and Pharmacy of Rabat (FMPR), Rabat-Morocco

Received: December 28, 2020; Accepted: January 21, 2021; Published: January 28, 2021

Abstract

Calcifying aponeurotic fibroma is a rare benign fibrous tumour with characteristic histopathological features commonly observed in children and adolescents. It usually involves the most distal part of the extremities. Despite being a benign tumour, it has a high risk of local recurrence. Our case is a 14-year-old child, attending school, followed for chronic renal failure with small kidneys who presented with a pathological fracture in his left big toe with a lytic X-ray image. Calcifying aponeurotic fibroma is a rare tumor, usually located in the subcutaneous layer, but it can manifest as a diffuse intra-articular calcified mass with numerous bone erosions. Calcification is absent at the early stage, hence the importance of careful radiological examination and complete histological analysis in order to rule out a probable malignant tumor. The rate of recurrence is important, hence the importance of surgical excision with healthy margins.

Keywords: Aponevrotic; Children; Antibodies; Fibroma; Toe

Introduction

Calcifying aponeurotic fibroma is a rare benign tumor (represents less than 1% of benign soft tissue tumors) and is most often found in children or young adults with a peak incidence between 8 and 14 years of age and sex. Ratio of 2 men to 1 woman (predominantly male). It develops, in 70% of cases, in contact with aponeuroses, fasciae and tendons of the palmar face of the hands. The second location in terms of frequency is the foot, especially the plantar surface. This tumor grows slowly, it remains painless, does not limit the course of the joint. Radiologically, the mass is poorly limited, with a heterogeneous signal on MRI. Calcifications are sometimes absent; in their presence they remain fine, punctate and of have mainly a central distribution. A biopsy is essential before accessing the excision in order to rule out the main differential diagnosis: synovialosarcoma.

Case Presentation

This is a 14-year-old child, in school, followed for chronic kidney disease with small kidneys. Currently he has microcytic hypochromic anemia, which has resulted in hair loss with significant inflammatory syndrome, all in a context of deterioration of his general condition. A pathologic fracture in the left toe revealed by swelling showed on the standard radiograph a well-defined lytic image with a periosteal reaction (Figure 1). A surgical biopsy was taken from the outgrowth and the fragments were sent to our laboratory. Two fragments were examined measuring respectively 0.3x0.3 and 0.3x0.2cm, they are completely included in a block. Histological study showed tumor proliferation made of elongated fibroblasts and epithelioids with oval or elongated nuclei with vesicular chromatin, the mitotic index is low estimated at mitosis by ten fields with Gx40. These fibroblasts are dispersed within a collagen-rich stroma, forming palisades around collagen nodules, which are sometimes calcified. PAS staining was performed with positivity in the collagen nodules. Inflammatory cells such as foamy histiocytes as well as bone sequesters are observed (Figure 2).