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PubMed1994

[Primary hemorrhagic spindle cell tumor of the lymph node with amianthoid structures. Case report and review of the literature].

An example of a benign haemorrhagic spindle cell tumour of the lymph node (synonym: intranodal myofibroblastoma) is presented, which was first described 5 years ago and has not as yet been documented in the German literature. This rare benign mesenchymal neoplasm of the lymph node occurs almost exclusively in the inguinal region. Diagnostic criteria include its composition of myofibroblastic cells (with immunoreactivity for vimentin and actin), which may show nuclear palisading, the presence of peripheral haemorrhage zones and the occurrence of characteristic amianthoid structures, which have the ultrastructural appearance of dense meshworks of collagen fibers. Knowledge of this tumour entity is important for differential diagnosis of primary and secondary malignant mesenchymal neoplasias of the lymph node.

Actin Cytoskeleton↗

PubMed2026

Pediatric intracranial inflammatory myofibroblastic tumor harboring DCTN1::ALK fusion: a case report with radiologic-pathologic-molecular correlation.

Central nervous system inflammatory myofibroblastic tumors are rare; pediatric DCTN1::ALK fusion cases are exceptionally uncommon. Here, we present an eight-year-old boy who presented with headache, vomiting, and a rapidly enlarging right frontal scalp mass. An MRI showed a dural, extra-axial lesion with mass effect. Histology confirmed IMT, and ALK immunohistochemistry was positive; next-generation sequencing (NGS) identified DCTN1 (exon 1-27)-ALK (exon 20-29) fusion, and FISH confirmed ALK rearrangement (33/100 nuclei). Genomic metrics showed tumor mutational burden (TMB) of 0.94/Mb, microsatellite stability, and CNV burden of 2.1%. He underwent near total resection followed by alectinib; to our knowledge, this is the first reported young pediatric (<10 years old) CNS IMT with this fusion.

Humans↗

PubMed2006

Recurrent gastric inflammatory myofibroblastic tumour in a 13-year-old male.

A 13-year-old boy who had epigastric pain and pallor for 2 months and found to have an ulcerative mass in the stomach and underwent partial gastrectomy. A diagnosis of inflammatory myofibroblastic tumour (IMT) of the stomach was made on histological examination. Three years later, recurrence in the stomach, with invasion into the pancreas and hilum of the spleen was noted and was managed by wide wedge resection of the stomach, distal pancreatectomy and splenectomy. The patient has been recurrence-free for the past 2 years. Gastric IMT is an uncommon tumour in children with unpredictable prognosis.

Adolescent↗

PubMed2006

Extramammary myofibroblastoma is genetically related to spindle cell lipoma.

Extramammary-type myofibroblastoma is a rare, benign spindle cell lesion, strictly resembling the breast counterpart, but occurring in extramammary sites, mainly in the inguinal/groin area. In this paper, we describe an extramammary-type myofibroblastoma in the groin of a 37-year-old male patient. The tumor showed a typical morphological and immunophenotypical profile, including staining for both CD34 and desmin. Dual-color interphase florescent in situ hybridization analysis revealed losses of RB/13q14 and FKHR/13q14 loci within tumor cells. The chromosome 13 rearrangements associated with the loss of the 13q14 chromosomal region are typically seen in spindle cell lipoma, and have been previously recognized in mammary myofibroblastoma, providing strong evidence for a pathogenetic link between these lesions.

Adult↗

PubMed2005

Inflammatory myofibroblastic tumor of the ileocecal mesentery mimicking abdominal lymphoma in childhood: report of two cases.

An inflammatory myofibroblastic tumor is an uncommon benign tumor located in various organs that can be misdiagnosed as a malignant neoplasm. We herein present two patients with ileocecal inflammatory myofibroblastic tumors. An abdominal mass was detected in a 13-year-old girl and a 15-year-old boy who presented with paleness, fatigue, intermittent fever, and night sweating. The radiological findings confirmed a mass originating from the ileocecal region. The presumptive diagnosis was Burkitt's lymphoma. The histopathological diagnosis was inflammatory myofibroblastic tumor. After a surgical resection, all systemic symptoms rapidly resolved. Inflammatory myofibroblastic tumor is a rare pseudosarcomatous clinical and pathological entity. Although this tumor is more commonly reported in the lung, it can be detected in extrapulmonary sites, including the mesentery. Because the choice of treatment for this tumor is conservative surgery, an accurate preoperative analysis is important to avoid any unnecessary aggressive surgical intervention or other therapeutic approaches.

Abdominal Neoplasms↗

PubMed1953