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Principles of Clinical Diagnosis
Published in Susan Bayliss Mallory, Alanna Bree, Peggy Chern, Illustrated Manual of Pediatric Dermatology, 2005
Susan Bayliss Mallory, Alanna Bree, Peggy Chern
Infantile myofibromatosis Seen at birth or early lifeMultiple cutaneous nodules with underlying tumors in the muscle, bone and visceral organsLesions are firm, skin-colored to red-purple nodules ranging in size from 0.5 to 7 cmIf limited to skin and bones, does not require treatment unless there is functional
The Giant Infantile Fibrosarcoma of Fetal Oropharynx and Anterior Neck
Published in Fetal and Pediatric Pathology, 2022
Tugba Sarac Sıvrıkoz, Lutfiye Selcuk Uygu, Çiğdem Kunt İşgüder, Erhan Aygun, Ibrahim Halil Kalelioglu, Recep Has
Fetal tumors are often detected as an incidental finding on prenatal sonography during the late second or third trimester. One-third of IFS cases are detected before birth, while the majority are diagnosed before the first 12 months [2]. Previously, Tadmor et al. [3] and Meizner et al. [6] reported prenatal IFS cases which were detected on the thigh and back at 22 and 23 weeks of gestation, respectively. According to our patient’s medical history, the mass was first detected at the 24th gestational weeks; unfortunately, there was no recorded image at that week. The current case was detected in a relatively early gestational week compared to other congenital soft tissue tumors detected in fetal life. It is the first prenatal case of IFS detected in the anterior neck originating from the tongue. Other soft tissue tumors should be considered in the differential diagnosis which may arise in this anatomical region, including teratomas, infantile myofibromatosis and infantile hemangiopericytoma [10]. Infantile hemangiopericytoma is superficially located and has a lobular rearrangement around vascular structures. There is an apparent differentiation in myofibroblasts and has typical zonal appearance in infantile myofibromatosis.
Myofibromatosis
Published in Fetal and Pediatric Pathology, 2021
Myofibromatosis is a distinctive mesenchymal disorder occurring predominantly in infancy and childhood. The term infantile myofibromatosis (IM) was first coined by Chung and Enzinger in 1981 [1]. Prior to this, the entity was designated with terms like congenital fibrosarcoma and congenital generalized fibromatosis [2]. Three forms of myofibromatosis have been recognized: i) solitary form, occurring in the skin and subcutis, ii) multiple form, with lesions in the skin, subcutis, skeletal muscle and bones, and iii) generalized form, with visceral lesions. A solitary tumor is known as myofibroma, while the multiple and generalized forms are termed as myofibromatosis [3]. There has been some debate in the literature regarding the origin and classification of myofibromatosis. Chung and Enzinger proposed the lesion to be of myofibroblastic origin based on ultrastructural features and some resemblance with fibromatosis [1]. Since then, categorization of myofibromatosis under the fibroblastic/myofibroblastic tumors has been widely accepted. However, WHO classification of soft tissue tumors, which is generally considered globally as a standard classification scheme, categorizes it under tumors of pericytic origin. It considers myofibroma to be a spectrum of myopericytoma [4]. Myofibromatosis needs to be differentiated from other childhood tumors like fibromatosis, lipofibromatosis, fibrous hamartoma of infancy and infantile fibrosarcoma. The diagnosis is essentially made on histopathology. The role of immunohistochemistry is mainly in differentiating it from its morphological mimickers. We present a case of myofibroma in the infraorbital region of an eleven-year-old male child and review its clinico-pathological features, differential diagnosis, and management.
Ex Utero Intrapartum Treatment (EXIT) in a rare infantile tongue fibrosarcoma and it’s management dilemma
Published in Acta Oto-Laryngologica Case Reports, 2023
Nabihah Roslle, Faizah Abdul Rahim, Norzi Gazali, Siti Sabzah Mohd Hashim, Goh Bee See
Imaging like CT scan or MRI may point to a solid mass but the exact diagnosis can only be made on histopathology examination powered by immunohistochemistry as seen in our case. Infantile fibrosarcoma can be graded into low, intermediate and high-grade depending on the severity of cellularity, nuclear pleomorphism and mitosis. Infantile fibrosarcoma must be kept in the differential diagnosis of soft tissue tumours like rhabdomyosarcoma and infantile myofibromatosis and its variants.