Solitary Infantile Myofibromatosis Research Articles

Magnetic Resonance Imaging Findings of Solitary Infantile Myofibromatosis of the Skull: A Case Report

Magnetic Resonance Imaging Findings of Solitary Infantile Myofibromatosis of the Skull: A Case Report

Solitary infantile myofibromatosis in the bones of the upper extremities: Two rare cases and a review of the literature

Infantile myofibromatosis (IM) is the most common fibrous tumor of infancy. IM may arise in a solitary or multicentric form, with similar histopathological findings, however, the clinical features and prognoses may vary. The solitary form tends to occur predominantly in males and is typically observed in the dermis, subcutis or deep soft tissues. The reported incidence of solitary osseous myofibromatosis is rare. Furthermore, the majority of solitary IM cases of the bone occur in the craniofacial bones, while the occurrence of solitary osseous myofibromatosis on the extremities has been sporadically reported. The present study describes two cases of solitary IM involving the bones of the upper extremities in females who were over two years old. The cases show unusual symptom presentation and the tumor origin is in a rarely observed location. The study discusses the clinical, radiological and pathological features, in addition to the previously described etiology, prognosis and treatment options for this condition.

Congenital solitary infantile myofibromatosis involving the spinal cord

Infantile myofibromatosis, a rare mesenchymal disorder that develops in early childhood, is classified by the number of lesions that occur: solitary or multicentric. Involvement of the CNS is unusual in either type. Infantile myofibromatosis in the spine is exceptional, and most published cases represent a secondary invasion. Here, the authors report on an 8-month-old girl presenting with weakness below the ankle and an intraspinal mass extending from T-6 to the conus. The patient underwent only partial surgical removal of the lesion, and the pathology was confirmed as infantile myofibromatosis. After the operation, weakness in the lower extremities gradually improved; however, she could not walk at the time of the final follow-up. On follow-up MRI performed 19 months after the operation, the residual lesion remained unchanged with decreased enhancement.

Solitary infantile myofibromatosis of the cranial vault: case report

Infantile myofibromatosis is a mesenchymal disorder of early childhood characterized by the formation of tumors in the skin, muscle, viscera, bone, and subcutaneous tissue. Although relatively rare overall, it represents the most common fibrous tumor of infancy. The etiology of this disorder is unknown. Infantile myofibromatosis can present as a solitary or multicentric form. With the multicentric form, bone is often involved, but solitary bone lesions account for only 10% of the cases. Imaging findings are not pathognomonic, and the differential diagnosis usually includes eosinophilic granuloma (Langerhans cell histiocytosis), osteomyelitis, metastasis, osteoblastoma, epidermoid cyst, hemangioma, fibrous dysplasia, fibrosarcoma, and meningioma. A histological pattern is typical, but there are no histopathological differences between the solitary and multicentric forms. Solitary lesions generally have a favorable prognosis if totally removed, with a 10% recurrence rate; incompletely resected lesions recur. We report the case of a 9-year-old boy who came to our attention with a solitary infantile myofibroma of the calvarium, appearing as a tight-elastic, lightly tender mass in the left frontal area, eroding both the inner and the outer tables. Histopathologically, the specimens showed a spindle-cell tumor with dense reticulin fiber network and expression of smooth muscle actin. Fifty-eight months MR follow-up after total removal showed no residual or relapse.

Infantile Myofibroma

This article presents a case report of a 37-week gestational age (GA) female infant (CK) whose first ultrasound at 35 weeks' GA revealed polyhydramnios, fetal ascites, and a possible diaphragmatic hernia. At birth, CK had a grossly distended abdomen, prominent abdominal veins, hepatomegaly, bounding femoral pulses, and generalized edema. Initial imaging identified an absent ductus venosus, absent segment of the inferior vena cava (IVC), and prominent superior vena cava to the right atrium. A computed tomography (CT) scan showed a mass contiguous with the liver causing compression of the IVC. Biopsy confirmed infantile myofibromatosis (IM), an uncommon soft tissue neoplasm that may present at birth or in early infancy. Although rare, this neoplasm is the most common fibrous tumor of infancy. The case of CK was unusual because the solitary IM lesion was in an atypical location; a solitary lesion is not commonly found in the viscera, and solitary lesions are predominant in males. Although lesions are often benign, visceral involvement is associated with high mortality. The cause is unknown, although familial cases have been reported. This article describes the key features of IM, possible treatment options, nursing care, and prognosis for infants with the disease.

Congenital occurrence of solitary infantile myofibromatosis of the spleen

Infantile myofibromatosis (IM) is a rare soft tissue tumor of infancy and childhood. We report the case of a newborn girl with an abdominal tumor discovered at 32 weeks of gestation by fetal ultrasound. She underwent a laparotomy for an unexplained abdominal mass 20 days after birth. The tumor originated from the spleen and was removed by splenectomy. There were no other abnormal findings on diagnostic modalities. Based on the histological examinations, the tumor was diagnosed as an IM. Although extremely rare during the neonatal period, solitary type IM should be considered as a differential diagnosis of the splenic tumor.

Solitary infantile myofibromatosis in the femur

We report on a case of the solitary type of infantile myofibromatosis of the right femur in an 11-month female. Radiographically, a well-defined osteolytic lesion was seen in the diaphysis of the right femur. Pathological study revealed that the spindle-shaped cells with eosinophilic cytoplasm were arranged in a fascicular and intertwining fashion. The cleft-shaped vascular spaces were observed between tumor nodules. Immunohistochemical staining caused many tumor cells to react for vimentin, alpha-smooth muscle actin and desmin. Ultrastructural study revealed numerous thin and intermediate types of filaments in the cytoplasm of the cells. To our best knowledge, this is the third reported case of solitary infantile myofibromatosis of long bones.

Infantile Myofibromatosis of the Skull

Infantile myofibromatosis is a rare benign tumor of childhood characterized by a myofibroblastic tumor in the skin, subcutaneous tissues, bones, and, occasionally, the viscera. The tumor may be solitary or multicentric. A solitary skeletal lesion most commonly occurs in the craniofacial region. We report a case of solitary infantile myofibromatosis of the skull, confirmed by excisional biopsy.

Solitary infantile myofibromatosis involving the clavicle

A rare case of solitary infantile myofibromatosis of bone is reported in the right clavicle of a 15-year-old boy. A radiograph demonstrated an osteolytic lesion with a sharp margin and a sclerotic rim. CT revealed a circumscribed lesion with slight expansion of the cortex. On MRI the lesion appeared isointense to muscle on T1-weighted images, bright on T2-weighted images, and showed marked gadolinium enhancement. The patient was well, without evidence of recurrence or metastasis, 4 years and 5 months following resection.

Infantile Myofibromatosis

Infantile myofibromatosis is a rare disease characterized by myofibroblastic proliferation, and typically occurs in early infancy. There is a wide spectrum of clinical presentation, which may involve various kinds of tissues in the body. Skin and subcutaneous lesions were the types of tissue most often seen. Although a multicentric form may behave aggressively, a solitary form of the tumor is benign with the possibility of spontaneous regression. Conservative management is justified after proper pathological diagnosis if the tumor involves an aesthetically important area. A case of solitary infantile myofibromatosis involving the upper lip is presented. Partial excision for biopsy was performed and long-term observation was undertaken. The tumor disappeared 3 years postoperatively.

Myofibromas of the oral cavity

Solitary infantile myofibromatosis or myofibroma of the oral cavity is an uncommon condition with only 32 reported cases in the English-language literature. This article presents four additional cases of these solitary myofibroblastic lesions. In addition, the clinical and histologic features of this uncommon spindle cell neoplasm have been reviewed. The similarity in both the clinical and histopathologic features of the "adult" and "infantile" lesions support the proposal that myofibroma is a more accurate and acceptable term for these solitary myofibroblastic lesions of the oral cavity.

Infantile Myofibromatosis: Report of Two Cases

Two cases of solitary type infantile myofibromatosis (IM) were presented. Case 1 was a 6-month-old male infant with a firm, dark red, fixed tumor on his right knee. A computerized tomographic scan revealed that the tumor was attached to the underlying muscle. Case 2 was a 1-month-old male infant with a tumor on his right knee, similar to that in case 1. In neither case was there any clinical evidence of visceral involvement. Histological and immunohistochemical findings were similar. The lesions appeared histologically as non-encapsulated nodules composed of whorled fascicles of spindle-shaped cells and a vascular element with a hemangiopericytoma-like appearance. The tumor cells were positively stained with PTAH. They were positive for alpha-smooth muscle actin and vimentin, but negative for desmin. These findings support the myofibroblastic nature of IM. In case 2, the tumor regressed spontaneously at the age of 12 months. Unlike the multicentric form, spontaneous regression of the solitary form of IM has not previously been reported.

Solitary Infantile Myofibromatosis of Bone

A rare case of solitary infantile myofibromatosis of bone in an 11-month-old boy is reported. Radiographically the lesion of parietal bone was round, well-circumscribed, and osteolytic with a sclerotic rim. Histologically the tumor was made up of nodules that were hyalinized or cellular and containing plump, spindle-shaped cells that were intermediate in appearance between fibroblasts and smooth-muscle cells, arranged in short bundles or whorls. Another typical feature was the presence of distended, cleft-shaped vascular spaces around the nodules. The microscopic features of this tumor were consistent with those of infantile myofibromatosis of other sites, such as the skin and deep soft tissue. The tumor cells showed immunoreactivities for vimentin and alpha-smooth muscle actin. Microfilaments with dense bodies were observed in the fibroblast-like tumor cells. In addition, many tumor cells stained for collagen type IV and were covered by incomplete external laminae, indicating infantile myofibromatosis has more advanced smooth-muscle differentiation than conventional fibromatosis.

Solitary infantile myofibromatosis of the mandible: Report of three cases

Infantile myofibromatosis (IMF) is a benign localized (solitary) or generalized (multicentric) proliferation of fibroblastic tissue occurring exclusively in infants and children. Three cases of solitary IMF involving the posterior region of the mandible of young children are reported. These lesions manifested clinically as asymptomatic bony expansion and roentgenographically as circumscribed lytic areas. Microscopically these tumors showed a distinct zoning phenomenon of curving bundles or intertwining fascicles of plump, spindle-shaped cells at the periphery and solid sheets of less differentiated round cells in the center. Positive immunostaining for vimentin and actin, with the lack of desmin and S- 100 protein reactivity, confirmed their myofibroblastic nature of these cells and supported the diagnosis of IMF. All three lesions were treated by curettage and the follow-up showed no incidence of recurrence or any other complications. As we demonstrate in these case reports, IMF should be included in the differential diagnosis of spindle cell neoplastic processes in children.

Solitary infantile myofibromatosis

Infantile myofibromatosis is an uncommon, benign, probably hamartomatous proliferation of myofibroblasts. Its growth is typically self-limiting but histologically it can simulate a sarcoma. A rare case of solitary infantile myofibromatosis presenting as an intraoral mass in a 10-year-old girl is reported.