Multimodal treatment of an extremely rare desmoplastic small round cell tumor primary to the brachial plexus - A case report and review of literature.

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignant neoplasm typically located in the abdomen or pelvis. Other possible locations are the chest, pleura, scrotum, and central nervous system. DSRCT originally arising from the brachial plexus (BP) is extremely rare, to the best of our knowledge, only two cases have been previously described in the English scientific literature. CASE DESCRIPTION: The authors present one new case of DSRCT arising from the left BP, the first in this location with rapid progression and in a female patient. We also highlight the importance of multimodal therapy, which included resection and both adjuvant radiation and chemotherapy. Macroscopic and microscopic characteristics of the lesion are detailed, as well as the patient's status at 56-month follow-up. CONCLUSION: For primary BP DSRCT, aggressive subtotal resection followed by radiation and chemotherapy can be satisfactory for disease control and for maintaining or improving the neurological status.

Mononeuropatía hipertrófica localizada: reporte de un caso y revisión de la literatura / Hypertrophic localized mononeuropathy: case report and bibliographic review

Objetivo: presentar un caso de mononeuropatía hipertrófica localizada. Descripción: paciente de 39 años de edad, que presenta una historia de dolor intenso y progresivo, hipoestesia en la región tenar y déficit motor en la mano izquierda. El examen físico mostró atrofia hipotener, mano en garra cubital y signo de Tinel en el antebrazo (8cm distal a la epitróclea). Intervención: la IRM mostró una masa a lo largo del recorrido del nervio cubital en el antebrazo. Se sugirió entonces tratamiento quirúrgico. No se obtuvo registro de conducción nerviosa durante la estimulación intraoperatoria, por lo cual se resecó la zona de fascículos afectados y se reemplazó la lesión por injertos autólogos extraídos de nervio sural. El diagnóstico histopatológico fue dificultoso y después de varias revisiones se concluyó que se trataba de de una mononeuropatía hipertrófica localizada. Conclusión: la paciente se encuentra actualmente bajo seguimiento y está mostrando signos de mejoría de la anestesía junto a remisión completa del dolor.

The authors report the case of a 39 year-old female, who presented with 10 years history of intense progressive pain, hypoesthesia in hypotenar region and motor deficit in the left hand. The physical exam showed hypotenar muscles atrophy, "claw hand", and Tinel’s sign in the forearm (8 cm distal from the medial epicondyle). Magnetic resonance imaging showed a mass lesion along ulnar nerve path in the forearm. Surgical treatment was, then, suggested. Nerve conduction could not be registered during intra-operative stimulation. Microsurgical resection of the damaged nerve fascicles and sural nerve grafting were performed. Histopathological diagnosis was difficult and could only be confirmed as localized hypertrophic mononeuropathy after revision of the biopsied material. The patient is being followed up and she is showing signs of hypoesthesia improvement in addition to complete remission of the pain.