Mesenteric lipoblastomatosis: an uncommon fat-containing childhood tumour diagnosed on imaging.

Lipoblastomatosis of mesentery is an extremely rare benign fat-containing mesenchymal tumour in infants and young children. On imaging, it appears as a solid infiltrating mass interspersed with macroscopic fat. We describe the distinctive imaging features of a large mesenteric Lipoblastomatosis, corroborated with intraoperative and histopathological features. We hope the case report and short review about this rare entity will increase the diagnostic confidence of reading radiologists while formulating differentials for lesions of similar appearance in the paediatric age group.

Lipoblastoma of the extremities.

BACKGROUND: Lipoblastoma is a rare benign tumor of infancy and childhood, with similar radiographic imaging findings as malignant liposarcoma. Therefore, it is crucial to distinguish through other diagnostics and clinical features. We present our experience with the diagnosis and clinical management of this entity. METHODS: A retrospective chart review was performed to identify children treated for lipoblastoma at our institution over a 30-year period. Charts were evaluated for diagnostic methods, treatment, and recurrence risk. RESULTS: A total of 9 males and 3 females were identified. The age at diagnosis ranged from 8 months to 44 years. Imaging modalities employed included ultrasound (N = 5), MRI (N = 12), X-ray (N = 8), and CT (N = 3). The tumor location was reported in the lower extremity (N = 8) and upper extremity (N = 4). The tumor size ranged from 1.7 to 18 cm (mean=7.08 cm). All patients underwent complete resection. Available pathology reports noted masses featuring mature adipocytes (N = 1), fibrous septa (N = 2), focal myxoid changes (N = 1), and spindle cells (N = 2). Nine patients underwent chromosomal analysis, which demonstrated five chromosome 8 abnormalities. No recurrences were reported. Three patients had follow-up imaging, and 11 had follow-up visits between 2 weeks to 15 years. CONCLUSION: Lipoblastoma is an uncommon childhood neoplasm of embryonic white fat that can also be present in adolescence and adulthood. Imaging, especially MRI, is helpful in limiting the differential diagnosis, but definitive diagnosis requires tissue biopsy, which should be obtained with core biopsy rather than fine needle aspiration. Surgical resection is curative with few complications. We do not recommend routine monitoring as recurrence is unlikely.

Paediatric thoracic wall lipoblastoma in a 7-year old: a rare tumour of childhood.

Lipoblastoma is a rare benign mesenchymal tumour derived from embryonic white fat tissue. Lipoblastoma generally occurs in children less than 3 years of age, and fewer than 200 cases have been reported in the English literature. There are hardly a dozen reports describing intrathoracic and thoracic involvement. We report a case of a 7-year-old boy who presented with a slowly growing right thoracic wall mass for a duration of 1 year. Radiological investigations (ultrasonography and contrast-enhanced CT of the chest showed the presence of a fat-containing, low density extrapulmonary soft tissue mass) could not confirm the diagnosis, and repeat biopsies also were not conclusive. The mass was excised and histopathology confirmed it as lipoblastoma. A review of literature pertaining to the clinical presentation, radiological features and histopathology of this rare condition has been discussed.

Lipoblastoma of scalp in a 4-month-old patient: an extremely rare case report and review of the literature.

Lipoblastoma is a rare benign tumor originating from adipose tissue, usually seen in infancy and early childhood. Here, we present a case of scalp lipoblastoma in a 4-month-old that we treated surgically and review the literature. Although lipoblastomas may be seen in various locations during infancy and early childhood, rarely, they can also develop in the scalp.

Large intrathoracic lipoblastoma.

Lipoblastoma is a rare benign tumor of primitive white fat in children, which usually involves the trunk and extremities. It rarely involves retroperitoneum, head, neck, and mediastinum. Herein, the authors introduce a rare presentation of lipoblastoma as a large intrathoracic mass.

Cardiac lipoblastoma of the pulmonary valve: A rare tumor in an unusual location.

BACKGROUD: Lipoblastoma is a rare, benign, fatty tissue tumour tumor that occurs in infancy and early childhood. Intrathoracic and mediastinal involvement of this tumour is rare, and an intracardiac location is even rarer. AIMS: To report the first case of a lipoblastoma in the pulmonary valve. MATERIALS: In this study, we report the case of a 15-month-old girl who presented with a tumour in the pulmonary valve. RESULTS: The tumour was completely resected macroscopically. Histological examination of the lesion confirmed the diagnosis of lipoblastoma. DISCUSSION: Most primary cardiac tumours are benign in the sense they are not invasive. However, benign tumours maintain the potential for serious illness related to significant haemodynamic compromise or life-threatening dysrhythmias. Due to the association of lipoblastoma with other heart malformations, preoperative diagnosis remains challenging. CONCLUSION: Tumours on the heart valve are more likely to cause haemodynamic disturbances. Complete resection yields an excellent prognosis.

Lipoblastomas and liposarcomas in paediatric patients: A case series.

Lipoblastomas and liposarcomas are rare causes of soft tissue masses in paediatric patients. In this retrospective clinical case series we identified 11 patients from our paediatric database (10 with a lipoblastoma and one with a liposarcoma) who had attended our hospital between 1998 and 2019. The median age of patients with lipoblastoma was 29 months. All lipoblastoma cases were managed with surgical excision and histological examination. The 18-year old patient with liposarcoma presented with a metastatic and unresectable tumour that was unresponsive to chemotherapy and radiation. Our experience demonstrates the importance of differentiating the type of soft tissue mass in children.

Imaging of head and neck lipoblastoma: case report and systematic review.

Lipoblastoma is a rare and benign tumour arising from embryonal fat cells, predominantly diagnosed in children younger than 3 years old. The most frequent locations are the extremities and trunk, while the head and neck areas are more rarely affected (10-15% of total cases). Clinically, the most common presentation is a fast-growing painless mass. Ultrasound is the first-line imaging examination, but Magnetic Resonance Imaging (MRI) allows for better definition of the relationships with the adjacent vascular and muscular structures. It can help to identify the lipomatous components, and it is useful for preoperative planning. However, the definitive diagnosis is provided by histopathological examination. Complete surgical excision is the first-line treatment, with a good prognosis in case of total eradication. We report the case of a 7-month-old male child with a rapidly growing mass that had typical radiological features of lipoblastoma.

Lipoblastoma: computed tomographic and magnetic resonance imaging features correlate with tumor behavior and pathology.

BACKGROUND: Lipoblastoma is a rare benign neoplasm of immature fat cells in children. Imaging appearances are frequently complex, sometimes simulating liposarcoma. OBJECTIVE: To characterize features of lipoblastoma on MRI and CT in comparison with recurrence risk. MATERIALS AND METHODS: We identified cases via retrospective review of histopathology-proven lipoblastoma cases in a large referral database and a pediatric medical center. Two radiologists scored CT and MRI on the basis of lesion features. RESULTS: We included a total of 56 children (32 boys and 24 girls) with a mean age of 2.6 years (range 0.1-13 years). Extremity lesions were most common (27%), followed by neck (19%), gluteal region (18%), chest (14%) and mesentery (14%). Children most commonly presented with painless masses (73%), followed by dyspnea (9%), distension (9%) and pain (7%). Non-adipose soft-tissue components were identified on CT and MRI in 78% of cases. Significant (moderate or marked) septations were noted in 59% and enhancement in 35%. Compartmental invasion was present in 43% of cases. Of paraspinal cases, 38% involved the neural foramina or central canal. Lesion complexity did not significantly correlate with age. Recurrence was observed in 9% of cases and was significantly correlated with compartmental invasion (correlation: 0.303, P=0.009) and septation complexity (correlation: 0.227, P=0.038) on initial imaging. CONCLUSION: Although lipoblastoma is a fat-containing entity, many lesions demonstrate marked complexity and local infiltration that resemble liposarcoma, which is exceedingly rare in younger children. Compartmental invasion and thicker septations appear to confer greater risk of recurrence following resection.

Mediastinal lipoblastoma in paediatrics. / Lipoblastoma mediastínico en pediatría.

OBJECTIVE: To describe two cases of mediastinal lipoblastoma, an infrequent and little-known patho logy, which is extremely rare in the mediastinum, with no cases reported in our country. CLINICAL CASES: Two case reports. Both patients were boys younger than three years, in which a mediastinal mass was found incidentally on a chest x-ray. The study was complemented with a CT scan and with a thoracoscopic biopsy in one of the cases. Complete resection of the tumor was achieved in both patients through thoracotomy. One of the patients presented Claude Bernard Horner syndrome as a complication from surgery, which resolved spontaneously after two years and the second case had no complications. CONCLUSION: Mediastinal lipoblastoma is a very rare pathology. Descriptions found in existing literature are similar to the cases presented in this article. We can conclude that mediastinal lipoblastomas in pediatrics present a very similar pattern and presentation, having a good prognosis if complete resection is achieved. Also, it is essential to distinguish it from its differential diagnoses in order to rule out malignancy.

Lipoblastoma mediastínico en pediatría / Mediastinal lipoblastoma in paediatrics

Resumen: Objetivo: Describir dos casos de lipoblastoma mediastínico, patología de baja incidencia y poco conocida, siendo aún más infrecuente en mediastino, sin haber reportes descritos en nuestro país. Casos Clínicos: 2 pacientes, ambos de sexo masculino, menores de 3 años, en los que se encontró como ha llazgo incidental una masa mediastínica en una radiografía de tórax. El estudio se complementó con una tomografía computada y en uno de los pacientes con una biopsia toracoscópica. En ambos se logró ex tirpación completa de la lesión vía toracotomía, siendo apoyado vía toracoscópica previa a la extracción de la lesión en uno de ellos. Uno de los pacientes evolucionó con Síndrome de Claude Bernard Horner el cual resolvió de manera espontánea luego de dos años y el segundo caso no presentó complicaciones. Conclusiones: el lipoblastoma mediastínico es una patología muy poco frecuente, las descripciones de la literatura se asemejan a los casos expuestos en este artículo, concluyendo que presentan un patrón muy similar entre ellos. Teniendo un pronóstico favorable si se logra la resección completa y siendo fundamental diferenciarlo de sus diagnósticos diferenciales para descartar malignidad.

Abstract: Objective: To describe two cases of mediastinal lipoblastoma, an infrequent and little-known patho logy, which is extremely rare in the mediastinum, with no cases reported in our country. Clinical Cases: Two case reports. Both patients were boys younger than three years, in which a mediastinal mass was found incidentally on a chest x-ray. The study was complemented with a CT scan and with a thoracoscopic biopsy in one of the cases. Complete resection of the tumor was achieved in both patients through thoracotomy. One of the patients presented Claude Bernard Horner syndrome as a complication from surgery, which resolved spontaneously after two years and the second case had no complications. Conclusion: Mediastinal lipoblastoma is a very rare pathology. Descriptions found in existing literature are similar to the cases presented in this article. We can conclude that mediastinal lipoblastomas in pediatrics present a very similar pattern and presentation, having a good prognosis if complete resection is achieved. Also, it is essential to distinguish it from its differential diagnoses in order to rule out malignancy.

Ultrasonography of Pediatric Superficial Soft Tissue Tumors and Tumor-Like Lesions.

Ultrasonography (US) is usually the first imaging examination performed to evaluate palpable or visible superficial soft tissue lesions that are common in children. Although clinical assessments, such as age at presentation, clinical course, and overlying skin discoloration, are important for the differentiation of pediatric soft tissue lesions, US allows a specific diagnosis of some typical benign lesions and helps in guiding further investigation since it provides detailed information about the lesion location, characterization including solid versus cystic, vascularity, and compressibility. Therefore, sufficient knowledge of the normal anatomy, proper ultrasonographic techniques, and the imaging findings of common and uncommon soft tissue lesions in children are crucial for accurate assessment and management of patients. In this article, we review the techniques and imaging findings focusing on the ultrasonographic features of a variety of superficial soft tissue lesions detected in children.

Robotic resection of recurrent pediatric lipoblastoma.

This case demonstrates successful resection of a rare, recurrent presacral-pelvic lipoblastoma in a 19-year-old female patient. Because of the anatomical location of the mass and its proximity to vital structures, the robotic approach allowed for both optimal visualization and effective debulking of the mass. Furthermore, with the use of an articulating laparoscopic camera, key visualization of the posterior lateral pelvis was possible. Using a wide breadth of technologies and resources is essential to broadening the surgical armamentarium and achieving resectability in otherwise challenging cases.

Rare retro peritoneal tumor in infants.

Lipoblastoma is a rare mesenchymal tumor, occuring in less than three-year-old infants in 90% of the cases and in 40% of the cases in chidren aged less than one year. It can be present at birth. It is a benign tumor, with defined margins, that can be locally aggressive, without metastases. We report the case of retroperitoneal lipoblastomatosis, with a neonatal diagnosis made by ultrasonographic and CT features.