CD34‐positive infantile myofibromatosis: Case report and review of hemangiopericytoma‐like pattern tumors

Abstract

We describe a case of CD34-positive infantile myofibromatosis with hemangiopericytoma-like pattern. A 2-day-old Japanese boy presented with multiple hemispherical nodules on the extremities and back. There was a biphasic histological growth in the dermis, accompanied by a hemangiopericytoma-like pattern with antler-like branching vessels. Tumor cells were oval to spindle-shaped myoid cells with bland appearance. Immunohistochemically, vimentin, calponin and CD34 were positive, while α-smooth muscle actin, h-caldesmon, HHF35 and desmin were negative. Although CD34 was positive, the present case could be diagnosed as infantile myofibromatosis. Myopericytoma, myofibroma/myofibromatosis, glomus tumor, glomangiopericytoma and angioleiomyoma share a continuous spectrum of benign hemangiopericytoma-like pattern tumors. Myofibroma/myofibromatosis is nearly included in myopericytoma among pericytic (perivascular) tumors, and could be positive for CD34. Several immunohistochemical panels of smooth muscle markers are needed for the diagnosis of pericytic (perivascular) tumors.

Diverse presentation and tailored treatment of infantile myofibromatosis: A single‐center experience

“…The patient had required antibiotic therapy for gram‐negative sepsis on the eighth day of life, and propranolol treatment for involvement of the tricuspid valve. A second biopsy from a soft tissue mass of the right upper back confirmed the diagnosis of a rare CD34‐positive generalized form of IM 5 . Chemotherapy with vinblastine and methotrexate was initiated at the age of 4 weeks.…”

Infantile myofibromatosis – a clinical and pathological diagnostic challenge

“…Immunohistochemical staining shows characteristics between fibroblasts and smooth muscle cells, being positive for vimentin and SMA; reactivity for desmin is variable [4]. CD-34 antigen has also been reported to be positive in cases of infantile myofibromatosis with hemangiopericytoma-like pattern [6].…”

“…It was only in 1981 that Chung and Enzinger introduced the term "infantile myofibromatosis" based on histochemical findings that allowed the authors to identify cell lines from which the tumor arises, further dividing this entity into subgroups with distinct prognosis depending on the location and involvement (with or without visceral lesions) [4]. More recently, IM has been considered to be part of a spectrum of tumors with perivascular myoid differentiation [5,6].…”

Stat6 Reliably Distinguishes Solitary Fibrous Tumors From Myofibromas

“…Furthermore, while CD34 reactivity is one of the hallmarks for SFT, 5-10% are reported to be negative, and 20-35% of tumors may show positivity for alpha-smooth muscle actin (αSMA) or epithelial membrane antigen (EMA) [1,4]. Rare cases of CD34 positivity in MF also have been reported [20]. While CD99 and Bcl-2 are helpful in diagnosis of SFT, they are non-specific, and tumors in its differential diagnosis may also demonstrate positivity to the same markers [21].…”