bims-myxlip 2025-04-13 papers

Issue of 2025–04–13
two papers selected by
Laura Mannarino, Humanitas Research

J Cancer Res Ther. 2025 Jan 01. 21(1): 299-302

Myxoid liposarcoma of thyroid in a 10-year-old child with Li-Fraumeni syndrome: A case report and review of literature.

Ankita Pandey, Pratibha Prashar, Renu Madan, Narendra Kumar.

ABSTRACT: Myxoid liposarcoma of the thyroid is an extremely rare entity seen mostly in the retroperitoneum, extremities, and trunk; however, its occurrence in the head and neck region is reported to be less than 2%. Due to its rarity, evidence-based management is lacking. Here we report a case of a 10-year-old girl with Li-Fraumeni syndrome who presented with a large mass in the thyroid. She underwent hemi-thyroidectomy followed by six cycles of chemotherapy with Ifosfamide and Adriamycin after the histopathologically confirmed diagnosis of myxoid liposarcoma. Within a year, she developed recurrent disease in supraclavicular region and mediastinum, causing superior vena cava obstruction. For this, she received palliative radiotherapy followed by excision of the residual mass and second line chemotherapy. However, the disease progressed and the patient succumbed to the disease within 19 months of diagnosis. In view of the rarity of the condition, the literature does not recommend any standard approach. Surgical excision is a standard first-line approach. As it is difficult to achieve extensive surgical margins in the neck region due to the presence of critical structures in the vicinity, postoperative radiation may improve local control rates.

DOI: https://doi.org/10.4103/jcrt.jcrt_1757_23

Pathologica. 2025 Feb;117(1): 39-44

Lipoblastoma-like tumor of the inguinal region: a close mimicker of myxoid liposarcoma.

Manuel Mazzucchelli, Giuseppe Angelico, Lucia Salvatorelli, Giada Maria Vecchio, Chiara Romano, Paolo Vigneri, Maria Paola Mariani, Gerardo Ferrara, Gaetano Giuseppe Magro.

Lipoblastoma-like tumor is a rare mesenchymal neoplasm, typically arising in the vulvar region of young women. Although it is considered a benign tumor, rare local recurrences and exceptionally distant metastases have been reported. Histological examination reveals a well-circumscribed tumor with lobulated pattern, composed of a mixture of mature adipocytes, spindle cells and lipoblasts set in abundant myxoid stroma with numerous thin-walled capillary-like vessels. Due to the rarity of this neoplasm and its morphological resemblance with other benign and malignant lipomatous tumors, the diagnosis of lipoblastoma-like tumor is often challenging. Herein, we present a case occurring in the inguinal region of a 28-year-old woman. Histological examination showed a mixture of mature adipocytes, bland-looking spindle cells with fibrillary cytoplasm, and numerous univacuolated lipoblasts set in a prominent myxoid matrix containing numerous thin-walled branching vessels. Immunohistochemically, neoplastic cells showed diffuse immunostaining for CD34 and negativity for α-smooth muscle actin, desmin, Rb1, MDM2 and STAT6. The main differential diagnoses included myxoid liposarcoma, spindle cell lipoma and cellular angiofibroma. FISH was negative for DDIT3; moreover, no evidence of regional gain or loss of RB1 was identified by FISH. Based on morphological, immunohistochemical and cytogenetic/molecular findings, a final diagnosis of "lipoblastoma-like tumor" of the inguinal region was rendered.

Keywords: RB1; lipoblastoma-like tumor; myxoid liposarcoma; soft tissue tumors; spindle cell lipoma

DOI: https://doi.org/10.32074/1591-951X-964