Infantile myofibromatosis

Larralde, Margarita; Ferrari, Bruno; Martínez, Juan Pablo; Barbieri, María Angélica Fernández; Méndez, José H; Casas, José Guadalupe

doi:10.1590/abd1806-4841.20175001

Cited by 19 publications

(29 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It is suggested that the underlying mechanism of tumor regression and growth is related to angiogenic stimulation and regression, both triggered by bFGF (basic Fibroblast-Growth-Factor) [6,7].…”

Section: Discussionmentioning

confidence: 99%

A rare case of neonatal-onset infantile myofibromatosis with metastatic recurrence in adulthood

Merlin¹,

Rouzic²,

Vignaud³

et al. 2018

Arch Clin Cases

View full text Add to dashboard Cite

Infantile myofibromatosis (IM) is a rare mesenchymal disorder, typically observed during infancy and characterized by the development of myofibroblastic tumors within skin, muscle, bone or viscera. In most cases, spontaneous regression of the lesions occurs before the age of four, however therapeutic tools such as surgery and chemotherapy sometimes need to be implemented. Metastatic recurrence of this condition is very rare. We report the case of a newborn infant with multicentric IM involving the skin, intestinal tract and bone, who required long-term symptomatic treatment. Spontaneous regression was noticed at the age of four but twenty years later she presented with a complete spontaneous right pneumothorax revealing cystic pulmonary metastases of IM. There have been very few reports of metastatic recurrence of IM in adulthood and this unique presentation underlines the need for long-term follow-up of these patients to detect and prevent possible complications.

show abstract

Section: Discussionmentioning

confidence: 99%

A rare case of neonatal-onset infantile myofibromatosis with metastatic recurrence in adulthood

Merlin¹,

Rouzic²,

Vignaud³

et al. 2018

Arch Clin Cases

View full text Add to dashboard Cite

show abstract

“…7 Imaging is useful in demonstrating the extent and progression of the tumor; however, it is not required in uncomplicated solitary cases. 4 Solitary and multicentric forms without visceral involvement have an excellent prognosis and often exhibit spontaneous regression. Symptomatic visceral involvement is associated with a mortality rate of 73%, mainly from cardiopulmonary or gastrointestinal tract complications.…”

Section: Discussionmentioning

confidence: 99%

“…No visceral or intracranial involvement was found, and the lesion involuted without therapy by the age of 10 months. 4 and 37% of multicentric cases have visceral involvement, which can affect any organ, such as the lung and heart, but most frequently the gastrointestinal tract. 4 Sporadic and familial cases have been reported.…”

mentioning

confidence: 99%

“…4 and 37% of multicentric cases have visceral involvement, which can affect any organ, such as the lung and heart, but most frequently the gastrointestinal tract. 4 Sporadic and familial cases have been reported. 3 Genetic sequencing of familial cases suggest an autosomal dominant (AD) inheritance pattern and platelet-derived growth factor receptor-β (PDGFRB) mutations as common features; however, IM is a genetically heterogeneous disease with incomplete penetrance and variable expressivity.…”

mentioning

confidence: 99%

“…There is a male predominance in the solitary form, and it affects the head and neck region more frequently. 4 In more than 50% of patients with solitary lesions and more than 90% with multiple lesions, the lesions are present at birth or soon after. In most of the remaining cases, lesions appear before the age of 2 years.…”

mentioning

confidence: 99%

See 2 more Smart Citations

Spontaneous Regression of an Infantile Scalp Tumor

2015

View full text Add to dashboard Cite

Solitary myofibroma preceding the development of multicentric myofibromatosis: A report of two cases with surveillance recommendations

Todd

Kim

Szabo

et al. 2020

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Infantile myofibroma is the most common fibrous tumor of infancy. Despite the frequency of these tumors, the natural history is incompletely understood. We present two cases with a unique pattern of disease: solitary myofibromas with subsequent progression to diffuse myofibromatosis. Given the variable spectrum of disease and the corresponding difference in morbidity and potential mortality based on the extent of disease, we propose surveillance recommendations.

show abstract

Infantile myofibromatosis

Cited by 19 publications

References 10 publications

A rare case of neonatal-onset infantile myofibromatosis with metastatic recurrence in adulthood

A rare case of neonatal-onset infantile myofibromatosis with metastatic recurrence in adulthood

Spontaneous Regression of an Infantile Scalp Tumor

Solitary myofibroma preceding the development of multicentric myofibromatosis: A report of two cases with surveillance recommendations

Contact Info

Product

Resources

About