RESUMO
Lipofibromatosis-like neural tumor (LPF-NT) is a rare variant of lipofibromatosis. Standard lipofibromatosis tumors show a predilection for the distal extremities of male children or young adults and are typically painless, slow-growing, subcutaneous or deep soft tissue masses. We present a case of a 50-year-old male with a slowly expanding, right foot mass. Physical examination revealed a painful, non-tender firm mass on the right medial foot. Magnetic imaging studies revealed a poorly defined soft tissue mass extending through subcutaneous tissue up to the dermis. Histologic examination revealed a spindle cell neoplasm. Immunohistochemistry showed co-expression of S100 protein, CD34 and TRK. In addition, the lesion was found to be positive for the LMNA-NTRK1 fusion by next-generation sequencing. These findings were supportive of a diagnosis of LPF-NT. At 3-month post-excision, the patient had no pain and repeat imaging indicated no evidence of tumor. The authors recommended including LPF-NT in the differential diagnosis of masses or lesions that are fibro-fatty tumors.
RESUMO
Testicular tumors account for 1-2% of all tumors in men, with 95% of these being germ cell tumors. Paraneoplastic limbic encephalitis is a rare sequela of testicular tumors associated with anti-Ma2 and KLH11 antibodies. The most effective treatment for paraneoplastic limbic encephalitis is treatment of the primary malignancy. We report a 41-year-old male that presented to the emergency department with episodic alteration of consciousness and memory disturbances. Negative neurologic evaluation and imaging led to concern for a paraneoplastic process from a distant malignancy. CT imaging revealed an enlarged, necrotic para-aortic lymph node and subsequent ultrasound demonstrated a right-sided testicular mass. Right radical orchiectomy was performed. Microscopically, the mass consisted of mixed respiratory epithelium, gastrointestinal glands, and squamous epithelium with keratinization consistent with a post-pubertal testicular teratoma with associated in situ germ cell neoplasia. Resection of the para-aortic mass revealed large anaplastic cells with epithelioid features, nuclear pleomorphism and frequent mitoses. Immunostaining was positive for Pan-Keratin and OCT4, consistent with poorly differentiated embryonal carcinoma. Resection of the primary and metastatic disease, as well as treatment with corticosteroids, resulted in resolution of the encephalitis. This presentation of severe neurological disturbances in the setting of a metastatic mixed non-seminomatous germ cell tumor represents a rare presentation of paraneoplastic limbic encephalitis.
