Intranodal Palisaded Myofibroblastoma: A Diagnostic Differential for Inguinal Lymphadenopathy

Patient: Male, 48-year-old Final Diagnosis: Intranodal palisaded myofibroblastoma Symptoms: Inguinal lump Medication: — Clinical Procedure: Lumpectomy Specialty: Pathology • Surgery OBJECTIVE: Rare disease BACKGROUND: Benign tumors of the lymph nodes are rare and are not usually considered in the differential diagnosis in cases of lymphadenopathy because reactive hyperplasia, lymphoma, and metastatic carcinoma are the most likely causes of enlarged nodes. Intranodal palisaded myofibroblastoma (IPM) is a very rare benign mesenchymal tumor of the lymph nodes most often affecting but not limited to the inguinal region, with up to 92 cases reported in the English literature. The cell of origin is the intranodal differentiated smooth muscle cell or myofibroblast. Although the pathophysiology of IPM remains unclear, theories about viral oncogenesis and mutational changes in the β-catenin gene with subsequent abnormal expression of β-catenin and cyclin D1 have been raised. CASE REPORT: We report a case of IPM in a 48-year-old man who presented with a mass in the left groin, with inconclusive imaging. The typical histologic findings of smooth muscle actin, cyclin D1, and β-catenin positive intranodal spindle cell proliferation with characteristic palisades, amianthoid fibers, collagenous bodies, lack of atypia, and very low mitotic count, together with characteristic profile on ancillary testing, confirmed the diagnosis. In addition to staining with smooth muscle actin, cyclin D1 and β-catenin, immunohistochemical studies showed focal positivity with desmin, a finding previously reported in 2 of the published cases. Surgical excision is usually curative, with a 6% recurrence rate and no reported cases of locally aggressive disease or malignant transformation. CONCLUSIONS: Although rare, IPM should be included in the differential diagnosis of isolated lymphadenopathy.