So-Called Congenital-Infantile Fibrosarcoma: Does It Exist and What Is It?

Abstract

Congenital-infantile fibrosarcoma (CIFS) is a cellular, mitotically active neoplasm with a paradoxically limited biologic potential in most cases. Its phenotype and proliferative features have been incompletely explored with inconclusive results. We studied the clinical, pathologic, immunohistochemical, and flow cytometric features of 26 cases (16 males, 10 females; 92% of cases detected within the first year life; 11 on extremities, 10 on the trunk, 5 in the head and neck). All displayed interlacing fascicles of spindle cells with focal necrosis, mitoses, and a focal hemangiopericytomatous vascular pattern. Immunohistochemically, 22 of 22 cases were reactive for vimentin. Other markers were present in a minority of cases. Flow cytometry of formalin-fixed, paraffin-embedded tissue in 10 cases demonstrated moderate to high proliferation activity and diploid DNA content in nine cases. Follow-up of all 26 patients revealed 20 patients alive and well, 15 without evidence of recurrence, and 5 with a small residual mass. Six patients had died of tumor, none had distant metastases. Patients with tumors in the head and neck or deep truncal soft tissues, including mesentery, had a poor prognosis because of local extension. CIFS is a clinically and morphologically homogeneous condition with considerable immunophenotypic diversity. Diploid DNA content in the majority of cases suggests that it may not be a fully expressed sarcoma. The clinicopathologic features are sufficiently distinctive to permit recognition and warrant conservative initial treatment in most cases. "Fibrosarcoma" is a term of convenience rather than of nosologic certainty.

Lumps and Bumps on the Head in Children: Use of CT and MR Imaging in Solving the Clinical Diagnostic Dilemma

“…Approximately 5% occur in the head and neck region (50 -52), and 25%-40% occur in children during the first 5 years of life, with up to 92% detected in the 1st year of life (53,54). The tumor is slightly more common in boys (60% of cases) (55).…”

Chest Wall Infantile Fibrosarcomas- A Rare Presentation

“…It is composed of malignant fibroblasts in a collagen background [1,2,4]. In contrast to the adult fibrosarcoma, IF is considered to be a rare and low grade malignant neoplasm.…”

“…Males are affected slightly more than females. Histopathologically, it should be differentiated from other tumors including infantile fibromatosis and myofibromatosis [1,2]. It is a highly cellular tumor, composed of spindle cells, which are arranged in bundles or fascicles.…”

“…Infantile fibrosarcoma (IF) is a rare malignant soft tissue neoplasm composed of uniform, cytologically malignant spindled fibroblasts [1,2]. It represents less than 1 % of all childhood cancers, and it is considered the most common soft tissue sarcoma in infants [3][4][5].…”

Directing oncogenic fusion genes into stem cells via an SCL enhancer

“…The developmental or stem cell origins of these cancers are unknown. CFS may have ambiguous or mixed cellular phenotypes (34), but the expression of vimentin and other markers (35,36) is believed to reflect a mesenchymal phenotype. In this context, it will be of interest to assess whether CFS TEL-TRKC can elicit any increased self-renewal in hemangioblasts and͞or their antecedent mesodermal precursors.…”