Maxillary Lipofibromatosis in a pediatric patient. Case report

This report discusses a rare case of a soft palate deformity in a young girl due to lipofibromatosis (LPF). This rare benign pediatric soft tissue tumour usually arises in the distal extremities. We believe this case represents the first report of lipofibromatosis involving only the maxillary bone.

Lipofibromatosis accompanied by several congenital anomalies, report of a rare case.

Lipofibromatosis is a rare tumor of infancy, which has been reported about 10 years ago, and since then very few reports have been published. This tumor has been reported in association with congenital anomalies such as macrosyndactyly, but there is no report of multiple congenital anomalies with lipofibromatosis in the English literature as far as our knowledge goes. Herein, we report a case of this tumor associated with syndactyly, bilateral complete cleft lip and palate, trigonocephaly, and atrial septal defect.

Lipofibromatosis: An unusual head and neck mass in the paediatric age group

Objective@#To describe a rare case of lipofibromatosis presenting as a head and neck mass in a 6-year-old child.@*Method@#Design: Case Report Setting: Tertiary Public General Hospital Patient: One @*Result@#A six-year-old male child admitted with a large right head and neck region mass underwent complete excision of a possible soft tissue neoplasm following investigations which included Fine Needle Aspiration Cytology, Ultrasonography and Computed Tomography. Histopathological examination yielded lipofibromatosis, a very rare lesion with a distinctive fibrofatty pattern. The patient was well with no recurrence after three months of follow up.@*Conclusion@#Although lipofibromatosis is a rare lesion in children and has a predilection for distal extremities, it may also present as a mass in the head and neck area. Complete surgical excision is feasible and is the only treatment option available for this rare lesion

Hamartoma fibroso de la infancia: informe de un caso / Fibrous hamartoma of infancy: case report

Introducción: El hamartoma fibroso de la infancia es una proliferación benigna de los tejidos blandos, de presentación infrecuente; 91 % de los casos ocurre durante el primer año de edad y afecta más frecuentemente al sexo masculino. Se caracteriza por ser una lesión subcutánea de morfología característica con patrón de crecimiento organoide trifásico. Caso clínico: Lactante masculino de seis meses de edad, con tumor en la región plantar medial del pie izquierdo, el cual fue resecado completamente. En el estudio histopatológico se informó como hamartoma fibroso de la infancia. Conclusiones: Puede localizarse en cualquier sitio anatómico, aunque 5 a 10 % afecta las extremidades inferiores existen pocos casos informados en el pie. En biopsias pequeñas es importante su diagnóstico diferencial con otras lesiones fibroadiposas para el adecuado tratamiento. La resección quirúrgica amplia con márgenes libres de lesión confiere un buen pronóstico a los pacientes.

BACKGROUND: Fibrous hamartoma of infancy (FHI) is an infrequent benign proliferation of the soft tissues. Ninety one percent of cases occur during the first year of life. FHI is characterized as a subcutaneous lesion with characteristic morphology with a triphasic organoid growth pattern. CLINICAL CASE: We present the case of a 6-month-old male infant with a tumor in the medial plantar region of the left foot, which was completely withered. Histopathological study reported a fibrous hamartoma of infancy. CONCLUSIONS: Fibrous hamartoma of infancy most frequently affects males. It may be localized at any anatomic site, although 5-10% of cases affect the lower limbs. Few cases are reported in the foot. This is a lesion with a characteristic morphological pattern; however, in small biopsies, its differential diagnosis is important with other fibroadipose lesions for appropriate treatment. Ample surgical resection with lesion-free borders confers a good prognosis for these patients.