Pediatric myofibroma/myofibromatosis of the soft tissue and bone: a clinicopathological analysis of 28 cases / 中华病理学杂志

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of pediatric myofibroma/myofibromatosis of the soft tissue and bone. Methods: All cases of pediatric myofibroma/myofibromatosis of the soft tissue and bone diagnosed between January 2011 and December 2018 were retrieved from the surgical pathology records in the Department of Pathology, Beijing Jishuitan Hospital, Beijing, China. Clinical and radiological data were collected. H&E and immunohistochemistry were used to examine histological and immunophenotypic features and to make the diagnosis and differential diagnosis. The relevant literature was also reviewed. Results: Twenty-eight cases of pediatric myofibroma/myofibromatosis of the soft tissue and bone were respectively collected. The patients' ages ranged from 2 months to 14 years, with a mean age of 7 years. There were 7 females and 21 males. There were 12 cases located in soft tissue, including the finger (n=9), upper arm (n=1) and foot (n=2). There were 14 cases located in the bone of limb, including the femur (n=8), tibia (n=4), clavicle (n=2), fibula (n=2) and radius (n=1). There were 2 cases of myofibromatosis involving multiple bones. Radiology showed lytic lesions in the bone. The proliferation of spindle-shaped myofibroblasts arranged in fascicles with indistinct eosinophilic cytoplasm and bland nuclei, with no pleomorphism and cytological atypia. The characteristic histologic structure was the biphasic nodular growth pattern with cellular and paucicellular regions. The tumors might arrange in a hemangiopericytoma-like pattern. The stroma varied between dense fibrosis and myxoid changes. The reactive new bone formation and inflammatory cell infiltration also existed. Immunohistochemical study showed that the SMA was positive. The surgical resections were performed. One of the patients had tumor recurrence as a result of 11-month follow-up. Conclusions: The pediatric myofibroma/myofibromatosis of the soft tissue and bone is a very rare benign tumor and has a good prognosis. It has a characteristic morphology and its differential diagnosis from other spindle cell tumors could be made with the immunohistochemical analysis.

Infantile myofibromatosis

Abstract: Infantile myofibromatosis is a mesenchymal disorder characterized by the fibrous proliferation of the skin, bone, muscle and viscera. It is the most common fibrous tumor in childhood. We present a newborn with skin and bone disease without visceral involvement, who showed good response to vinblastine and methotrexate. Clinical features, etiology, diagnosis, and treatment are reviewed.

Miofibromatose infantil: relato de uma rara doença / Infantile myofibromatosis: report of a rare disease

A miofibromatose infantil é uma rara doença que tem várias formas de apresentação. Habitualmente, manifesta-se com nódulos subcutâneos, que podem ou não estar associados à presença de nódulos viscerais. Deve-se estar apto a fazer o diagnóstico por meio do exame físico e de imagem, que evidenciarão o padrão das lesões para estadiar/classificar a doença. O tratamento ainda é controverso.

Infantile myofibromatosis is a rare disease with various presentations. Usually, such condition manifests itself as subcutaneous nodules, either in association or not with visceral nodules. The diagnosis should be achieved by means of physical examination and imaging studies, with emphasis on the lesions pattern to allow the staging and classification of the disease. The treatment is still controversial.

Possibility of Alternative Medicine in the Field of Pediatric Hematology-Oncolgy: Analysis of 7 Cases of Unexpected Outcome by Modern Medicine / 임상소아혈액종양

BACKGROUND: We analyzed data for pediatric hematology-oncology patients who had unexpected outcomes after they received alternative medicinal treatment; these outcomes could not be explained by modern medicine.METHODS: The medical history of 7 of the pediatric patients who were treated at Seoul National University Children's Hospital from 1987 to 2011 and had unexpected outcomes was analyzed retrospectively.RESULTS: The first patient had recurrent infantile fibrosarcoma and had more than 10 multiple coin lesions in both the lungs at the time of the third relapse. He received acupuncture twice weekly for 10 years without tumor progression. The second patient had recurrent fibromatosis in the neck and caused compression of the trachea and heart. The third patient had a malignant peripheral nerve sheath tumor and the fourth patient had inoperable inflammatory myofibromatosis. The second, third, and fourth patients were treated using a herbal medication that contained Phellinus linteus extract and exhibited responses ranging from partial to stable disease. The fifth and sixth patients had multiple focal nodular hyperplasia of the liver and recurrent renal cell cancer. These 2 patients exercised strict dietary control and underwent an exercise program that were adjusted according to their body constitution and were based on Sasang typology. These 2 patients now have stable disease. The seventh patient had unresponsive chronic idiopathic thrombocytopenic purpura and showed complete recovery of the platelet count after cepharanthine treatment.CONCLUSION: Scientific investigations are required to prove the effectiveness of these alternative medicines and to improve the results of such treatment in pediatric patients.

Possibility of Alternative Medicine in the Field of Pediatric Hematology-Oncolgy: Analysis of 7 Cases of Unexpected Outcome by Modern Medicine / 임상소아혈액종양

BACKGROUND: We analyzed data for pediatric hematology-oncology patients who had unexpected outcomes after they received alternative medicinal treatment; these outcomes could not be explained by modern medicine. METHODS: The medical history of 7 of the pediatric patients who were treated at Seoul National University Children's Hospital from 1987 to 2011 and had unexpected outcomes was analyzed retrospectively. RESULTS: The first patient had recurrent infantile fibrosarcoma and had more than 10 multiple coin lesions in both the lungs at the time of the third relapse. He received acupuncture twice weekly for 10 years without tumor progression. The second patient had recurrent fibromatosis in the neck and caused compression of the trachea and heart. The third patient had a malignant peripheral nerve sheath tumor and the fourth patient had inoperable inflammatory myofibromatosis. The second, third, and fourth patients were treated using a herbal medication that contained Phellinus linteus extract and exhibited responses ranging from partial to stable disease. The fifth and sixth patients had multiple focal nodular hyperplasia of the liver and recurrent renal cell cancer. These 2 patients exercised strict dietary control and underwent an exercise program that were adjusted according to their body constitution and were based on Sasang typology. These 2 patients now have stable disease. The seventh patient had unresponsive chronic idiopathic thrombocytopenic purpura and showed complete recovery of the platelet count after cepharanthine treatment. CONCLUSION: Scientific investigations are required to prove the effectiveness of these alternative medicines and to improve the results of such treatment in pediatric patients.

Myopericytoma-type Perivascular Myoma Arising on the Auricle of the Ear / 대한피부과학회지

The concept of perivascular myomas was recently proposed to designate perivascular myoid tumors arising from pleuripotent perivascular cells like smooth muscle cell cells, pericytes and glomus cell. They represent a histological continuum between three categories; myofibromatosis, glomangiopericytoma and myopericytoma. Myopericytoma is a rare, soft tissue tumor that primarily occurs in the skin and the subcutaneous and soft tissues of the extremities, but very few cases have been reported in other locations, which occurred in our present case. We herein report on a rare case of myopericytoma that developed on an unusual location, that is, the ear. The patient was a 35-year-old man who had a painless, slowly-growing red nodule on the upper auricle of the right ear for 5 years. The nodule was revealed to be a well-circumscribed mass composed of round to oval cells that strongly expressed smooth muscle actin. These cells were arranged in the concentrically multilayered pattern and they were surrounded by small to medium-sized vessels.

A case report of infantile myofibromatosis of left mandibular angle / 华西口腔医学杂志

The clinical data of one case of infantile myofibromatosis of left mandibular angle were analyzed, and the clinicopathological characteristics, imaging diagnosis, treatment and prognosis of infantile myofibromatosis were discussed.

Miofibroma congénito. un hemangiopericitoma verdadero: un caso neonatal con estudio inmunohistoquímico y ultraestructural / Congenital myofibroma: a true hemangiopericytoma: a neonatal case with immunohistochemical and ultrastructural studies

Se presenta el caso de un neonato masculino con tumor dérmico pedículado, localizado en la región malar derecha; sometido a resección quirúrgica completa con evolución postoperatoria satisfactoria. Los hallazgos histopatológicos revelaron un tumor subcutáneo de aspecto nodular con crecimiento intravascular subendotelial, constituido por una población dual de células pequeñas y células fusiformes, distribuidas en un patrón bifásico. Todas las células tumorales mostraron una fuerte reacción pericelular al PAS. Los estudios inmunohistoquímicos expresaron: positividad citoplasmática difusa para CD34 y Vimentina en todas las células tumorales, y positividad citoplasmática para la a-actina del músculo liso solo en las células tumorales fusiformes y aisladas células neoplásicas menos diferenciadas. La microscopía electrónica demostró membrana basal y en el citoplasma, numerosos filamentos intermedios con condensación focal. Todos estos hallazgos nos permiten concluir, se trata de un miofibroma, un hemangiopericitoma verdadero con diferenciación miofibroblástica. Por esta razón, proponemos el término miofibropericitoma, para resaltar su origen pericítico y su diferenciación miofibroblástica. Enfatizamos la necesidad de reconocer esta entidad, dada su baja frecuencia y la posibilidad de error diagnóstico con otros tumores de tejidos blandos que exhiben hallazgos parecidos al hemangiopericitoma.

Miofibromatosis infantil / Infantile myofibromatosis

La miofibromatosis infantil es una enfermedad del período neonatal o de la infancia temprana; consiste en la aparición de uno o múltiples tumores fibrosos que afectan piel, tejido celular subcutáneo, músculos, huesos y/o vísceras. Son tumores con tendencia a la involución espontánea, y su pronóstico está condicionad por la presencia o no de compromiso visceral. Se presenta el caso de un recién nacido de 48 horas de vida, con una tumoración en miembro superior derecho, sin compromiso visceral, que presentó involución espontánea en un plazo de 8 meses.

Infantile myofibromatosis is an illness of the neonatal period or early childhood. It consists of the appearance of one or more fibrous tumors which affect the skin, subcutaneous tissue, muscles, bones and/or viscera. These tumors tend to undergo spontaneousinvolution, and the prognosis is conditioned by the presence or absence of visceral liability. The case of a 48-hour newborn is described. The infant presented a tumour in the upper right member, which did not revealedvisceral liability and which resulted in spontaneous involution over an eight month span.

A Case of Solitary Adult Type Myofibroma / 대한피부과학회지

Solitary myofibroma is a recently described, uncommon neoplasm of superficial soft tissue and presenting as an asymptomatic nodule of the skin, which mainly occurs in adults. It was thought to be the adult counterpart of infantile myofibromatosis. Although it has histopathological similarity to infantile myofibromatosis, it shows a discrete clinicopathological entity compared with infantile form on the basis of its much later onset, superficial location, invariable solitary occurrence and uniformly benign behavior. A 41-year-old female presented a skin lesion on the posterior neck for 1 year, which was a solitary, painless nodule. The excised tumor was diagnosed as myofibroma by light microscopy and immunohistochemistry.

A Case of Infantile Myofibromatosis / 대한피부과학회지

Infantile myofibromatosis is the most common of the juvenile fibromatoses and an unusual mesenchymal disorder characterized by the proliferation of tumors in the skin, muscle, bone and viscera. There are two forms of the disease: solitary and multicentric. A benign course with spontaneous regression is seen in most patients, but poor prognosis can be associated with the multicentric form, in which visceral organs may be involved. It usually presents in early childhood. We report a case of infantile myofibromatosis arising in the left sole of a female new born. Physical examination showed a firm, red colored 2x4cm sized tumor. Histological examination revealed a spindle cell tumor in the dermis with fascicles of plump spindle cells with a whorled arrangement.

A Case of Solitary Adult Type Myofibroma in Eyelid / 대한피부과학회지

Solitary myofibroma is an uncommon myofibroblastic neoplasm, presenting as an asymptomatic nodule of the skin, which mainly occurrs in adults. It was thought to be the adult counterpart of infantile myofibromatosis. Although it has histopathological similarity to infantile myofibromatosis, it shows a discrete clinicopathological entity compared with infantile form on the basis of its much later onset, superficial location, invariable solitary occurrence and uniformly benign behavior. A-45-year-old woman had a skin lesion on the right upper eyelid for 2 years, which was a solitary, painless nodule with eyelid retraction. The excised tumor was diagnosed as myofibroma by light microscopy and immunohistochemistry.

A Case of Solitary Myofibroma In Adult / 대한피부과학회지

Solitary myofibroma is a recently described, benign neoplasm of superficial soft tissue, which represents the adult counterpart of infantile myofibromatosis. In Korean literature, only one case has been reported, and we report an additional case of solitary myofibroma in a 57-year-old man. He presented with 2-month history of asymptomatic slightly elevated pale-skin-colored nodule occurring on the right side of philtrum. A histopathologic examination revealed diffuse proliferation of fibroblasts and spindle cells with a whorled arrangement and grouped fascicles. The tumor cells were positively stained for vimentin and smooth muscle actin.

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.

Infantile Myofibromatosis of the Skull: Case Report

No abstract available.

A Case of Multicentric Infantile Myofibromatosis / 대한소아혈액종양학회지

Infantile myofibromatosis is one of the most common fibrous proliferation of infancy, characterized by the development of non-tender, firm, discrete, flesh-colored to purple nodules in skin, muscle, bone, and subcutaneous tissues. Three distinct subtypes are recognized-solitary, multicentric and generalized subtype. In solitary and multicentric subtypes, the prognosis is excellent with expected spontaneous regression of nodules in 1 to 2 years. But the prognosis of generalized subtype is poor with 75% of patients dying within the first few months of life secondary to cardiopulmonary or gastrointestinal complications. We report a case of multicentric infantile myofibromatosis in a 4-year-old boy presenting with multiple, asymptomatic, fixed and protruding masses on the posterior occiput, shoulders, upper and lower back and right lateral chest wall. Over the subsequent 20 monthes, the child developed limitation of motion of back and shoulder with the repeated formation and disappearance of myofibromatosis of the skin and musculature, but without visceral involvement.