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Journal of Neonatal Surgery 2012
PubMed: 26023394
DOI: No ID Found -
Auris, Nasus, Larynx Dec 2020Infantile myofibromatosis is a rare condition characterized by benign spindle cell tumors most commonly involving the head, neck, and chest. An infant female with a...
Infantile myofibromatosis is a rare condition characterized by benign spindle cell tumors most commonly involving the head, neck, and chest. An infant female with a prenatal diagnosis of a large facial mass was delivered via Cesarean at 34 weeks. Sparse prenatal care was received. Following delivery, the neonate was found to have an 8 cm ulcerative mass involving the upper lip and philtrum. Respiratory distress developed, and mask ventilation was difficult secondary to the size of the mass. The patient was successfully intubated after numerous attempts and then transferred to the children's hospital. Additional imaging demonstrated similar masses within bilateral iliopsoas and gluteal muscles, and her right gastrocnemius. A biopsy confirmed infantile myofibromatosis. At two weeks of life, she underwent resection with bilateral myocutaneous advancement flaps and successful extubation. She received adjuvant vinblastine and methotrexate for her pelvic and extremity disease with excellent response. We present the first case of airway distress secondary to myocutaneous myofibromatosis.
Topics: Female; Humans; Infant, Newborn; Magnetic Resonance Imaging; Myofibromatosis; Nasal Obstruction; Respiratory Insufficiency
PubMed: 31677854
DOI: 10.1016/j.anl.2019.10.005 -
European Radiology 2000We present a case of solitary infantile myofibromatosis of the skull in a 3-month-old boy. A right parietal subcutaneous lump was found at birth, and it increased in...
We present a case of solitary infantile myofibromatosis of the skull in a 3-month-old boy. A right parietal subcutaneous lump was found at birth, and it increased in size over the ensuing 3 months. Surgery was performed, and a diagnosis of myofibromatosis was confirmed histopathologically. Solitary myofibromatosis of the skull is extremely rare. The radiographical, CT, and MR appearances, as well as histopathological findings, are described in this article.
Topics: Humans; Infant; Leiomyoma; Magnetic Resonance Imaging; Male; Skull Neoplasms; Tomography, X-Ray Computed
PubMed: 10663739
DOI: 10.1007/s003300050028 -
The Journal of Craniofacial Surgery Jun 2015
PubMed: 26080220
DOI: 10.1097/SCS.0000000000001598 -
Pediatric Blood & Cancer Jun 2020Infantile myofibromatosis (IM) is characterized by solitary musculoskeletal nodules presenting during infancy but can manifest as multiple lesions with visceral...
Infantile myofibromatosis (IM) is characterized by solitary musculoskeletal nodules presenting during infancy but can manifest as multiple lesions with visceral involvement. Multicentric IM with visceral involvement carries a high risk of mortality and there is no consensus on treatment. We present a case of a patient with multicentric IM and pulmonary involvement who progressed on several chemotherapeutic regimens and subsequently had a complete response to sorafenib and later imatinib. This report describes the novel use of sorafenib and imatinib to treat generalized IM and the role of continued tyrosine kinase inhibitor therapy to maintain remission.
Topics: Antineoplastic Combined Chemotherapy Protocols; Female; Humans; Imatinib Mesylate; Infant; Myofibromatosis; Prognosis; Sorafenib
PubMed: 32307894
DOI: 10.1002/pbc.28288 -
Journal of Otolaryngology - Head & Neck... Mar 2019Infantile myofibromatosis is the most common benign fibrous tumor in infants. Three different types have been reported in the literature. The most commonly affected...
BACKGROUND
Infantile myofibromatosis is the most common benign fibrous tumor in infants. Three different types have been reported in the literature. The most commonly affected areas are the head, the neck and the trunk. Our patient showed a very high level of mandibular destruction resistant to all mandibular sparing treatment strategies requiring segmental mandibulectomy and complex reconstruction.
CASE PRESENTATION
We describe a rare case of multicentric infantile myofibromatosis with mandibular bone destruction. The treatment required a succession of chemotherapy, a subtotal transoral resection and a hemi-mandibulectomy. The mandibular reconstruction was staged with initial bridging titanium plate with a submental flap, followed later by a fibula free flap.
CONCLUSION
Mandibular involvement by myofibromatosis is rare, and the extend of bone destruction and reconstruction make this case unique. To our knowledge, this is the only reported case of fibula free flap mandibular reconstruction in a patient with infantile myofibromatosis , as well as one of the youngest reported submental island flaps for any pathology. We describe the clinical presentation and management, including relevant imaging, histopathology, medical and surgical treatment as well as a review of relevant literature.
Topics: Free Tissue Flaps; Humans; Infant; Male; Mandibular Osteotomy; Mandibular Reconstruction; Myofibromatosis; Plastic Surgery Procedures
PubMed: 30871614
DOI: 10.1186/s40463-019-0333-z -
JCO Precision Oncology Oct 2022
Topics: Carcinogenesis; Drug Monitoring; Humans; Imatinib Mesylate; Myofibromatosis; Receptor, Platelet-Derived Growth Factor beta
PubMed: 36201717
DOI: 10.1200/PO.22.00250 -
Revue de Stomatologie Et de Chirurgie... Nov 2007Pediatric myofibromatosis is a rare tumor in neonates and children. Two forms are described, solitary and multicentric, the solitary type is more common and is localized...
INTRODUCTION
Pediatric myofibromatosis is a rare tumor in neonates and children. Two forms are described, solitary and multicentric, the solitary type is more common and is localized mainly on the head and the neck, mandible involvement is rare. The aim of this article was to report the anatomoclinical and therapeutic features of this pediatric tumor in a case as well as its follow-up.
CASE REPORT
A 10-year-old girl was brought to consultation for a lower left gingival swelling 5 cm in diameter, forming a unit with the mandibular bone. The volume had gradually increased over the last 12 months. Imagery revealed the presence of an osteolytic tumor benign in aspect, but locally aggressive. Conservative surgery was performed. The diagnosis of pediatric myofibromatosis was confirmed. Evolution was excellent and after three years of follow-up, there was no evidence of relapse.
DISCUSSION
Pediatric myofibromatosis often presents as a painless, well-circumscribed, solid nodule. Imagery is very useful to assess lesion extension and for the therapeutic follow-up. The diagnosis is made on anatomopathological findings and immunohistochemical assessment. The treatment of the solitary myofibromatosis is primarily surgical and its prognosis is excellent contrary to the multicentric form.
Topics: Actins; Child; Female; Histocytochemistry; Humans; Mandibular Neoplasms; Myofibroma; Tomography, X-Ray Computed; Vimentin
PubMed: 17632191
DOI: 10.1016/j.stomax.2006.11.009 -
Dermatology Online Journal Apr 2017Infantile myofibromatosis is a rare disorder of fibroblastic/myofibroblastic proliferation and represents the most frequent type of mesenchymal tumor in the neonatal...
Infantile myofibromatosis is a rare disorder of fibroblastic/myofibroblastic proliferation and represents the most frequent type of mesenchymal tumor in the neonatal period and primary infancy.Three clinical types have been described: solitary, multicentric, and generalized (with visceral involvement). A correct characterization of the histopathology is essential to diagnose these neoplasias in early infancy. We present a case of multicentric infantile myofibromatosis with regression over time.
Topics: Female; Humans; Infant; Myofibromatosis; Neoplasm Regression, Spontaneous
PubMed: 28541875
DOI: No ID Found -
Journal of the European Academy of... Feb 2008
Topics: Humans; Infant, Newborn; Male; Myofibromatosis; Prognosis; Remission, Spontaneous; Skin
PubMed: 18211420
DOI: 10.1111/j.1468-3083.2007.02292.x