Hybrid neurofibroma/schwannoma of the orbit / Tumor híbrido neurofibroma/schwannoma de la órbita

Hybrid neurofibroma/schwannoma is a rare variant of hybrid peripheral nerve sheath tumours (HPNST). A Medline search up to December 2021 identified only six cases of this tumour in the orbit. We report the case of a 78-year-old man who presented with left exophthalmos. Computed tomography showed a left intraconal orbital mass. The clinico-radiological diagnosis was consistent with an intraconal cavernous angioma. Orbitotomy was performed, obtaining an 18×16×11mm mass. Two different morphologies were seen microscopically, diagnostic of hybrid neurofibroma/schwannoma. HPNSTs of the orbit are uncommon and most reported cases showed a hybrid neurofibroma/schwannoma morphology. Hybrid neurofibroma/schwannomas have been associated with neurofibromatosis and schwannomatosis. Local recurrences have been reported. The correct identification of these tumours is important due to their potential use as a syndromic marker.(AU)

El tumor híbrido neurofibroma/schwannoma es una lesión rara incluida dentro de tumores híbridos de la vaina del nervio periférico. Mediante revisión bibliográfica en Medline hasta diciembre de 2021 se identificó solo seis casos de este tumor en la órbita. Presentamos el caso de un varón de 78 años que consultó por exoftalmos izquierdo. La tomografía computarizada mostró una masa orbitaria intraconal izquierda. El diagnóstico clínico-radiológico fue compatible con angioma cavernoso intraconal. Se realizó una orbitotomía, obteniendo una masa de 18×16×11mm. Microscópicamente, el tumor mostró dos morfologías diferentes compatibles con tumor híbrido neurofibroma/schwannoma. Los tumores híbridos de la vaina del nervio periférico de la órbita son poco comunes. La mayoría de los casos informados muestran una morfología híbrida de neurofibroma/schwannoma. El tumor híbrido neurofibroma/schwannoma se ha asociado con neurofibromatosis y schwannomatosis. Se han reportado recurrencias locales. La correcta identificación de estos tumores es relevante debido a su potencial uso como marcador sindrómico.(AU)

Hybrid neurofibroma/schwannoma of the orbit.

Hybrid neurofibroma/schwannoma is a rare variant of hybrid peripheral nerve sheath tumours (HPNST). A Medline search up to December 2021 identified only six cases of this tumour in the orbit. We report the case of a 78-year-old man who presented with left exophthalmos. Computed tomography showed a left intraconal orbital mass. The clinico-radiological diagnosis was consistent with an intraconal cavernous angioma. Orbitotomy was performed, obtaining an 18×16×11mm mass. Two different morphologies were seen microscopically, diagnostic of hybrid neurofibroma/schwannoma. HPNSTs of the orbit are uncommon and most reported cases showed a hybrid neurofibroma/schwannoma morphology. Hybrid neurofibroma/schwannomas have been associated with neurofibromatosis and schwannomatosis. Local recurrences have been reported. The correct identification of these tumours is important due to their potential use as a syndromic marker.

Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome.

INTRODUCTION: We aim to investigate the clinical onset, computed tomography (CT) and magnetic resonance (MR) imaging findings, and follow-up of patients with cerebral amyloid angiopathy (CAA)-related inflammation, an uncommon but clinically striking presentation of CAA. METHODS: We retrospectively reviewed the clinical manifestations, CT/MR imaging findings, and outcome of ten consecutive patients with CAA-related inflammation. In each patient, a brain CT study was performed at hospital admission, and brain MR imaging was carried out 2 to 4 days later. Clinical and radiologic follow-up findings were evaluated in all patients. RESULTS: The most common clinical onset was rapidly progressive cognitive decline, followed by focal neurological signs. Brain CT/MR showed unenhanced expansive subcortical lesions, corresponding to areas of vasogenic edema, associated with chronic lobar, cortical, or cortical-subcortical micro/macrohemorrhages. Clinical symptoms recovered in a few weeks under treatment in eight patients and spontaneously in the remaining two. MRI follow-up at 2 to 12 months after treatment showed resolution of the lesions. Three patients experienced symptomatic disease recurrence, with new lesions on CT/MR. CONCLUSION: In the absence of histological data, early recognition of the clinical symptoms and typical radiologic features of CAA-related inflammation is essential to enable timely establishment of proper treatment.

MDCT in the preoperative planning of abdominal perforator surgery for postmastectomy breast reconstruction.

OBJECTIVE: This study aimed to evaluate the utility of MDCT in planning abdominal perforator surgery for breast reconstruction in patients who have undergone mastectomy. SUBJECTS AND METHODS: One hundred twenty-six consecutive patients scheduled for postmastectomy breast reconstruction using deep inferior epigastric perforator flaps underwent MDCT. The images were evaluated to identify, characterize, and map the dominant musculocutaneous perforator vessels of the deep inferior epigastric artery. In the first 36 patients, we compared the intraoperative findings with the preoperative MDCT findings. In the latter 90 patients, the dominant perforator vessels were directly selected on the basis of MDCT findings. RESULTS: We found an exact correlation between the intraoperative and radiologic findings in the first 36 cases. In the following 90 cases, the average operating time saved per patient was 1 hour 40 minutes and there was a significant reduction in postsurgical complications. The preoperative evaluation by MDCT confirmed the wide range of variability in the vascular anatomy of the abdominal wall previously described in anatomic studies. CONCLUSION: MDCT provides valuable information before surgery about the arterial anatomy of the inferior abdominal wall. It enables accurate identification of the most suitable dominant perforator vessel and makes surgical perforator flap procedures for breast reconstruction faster and safer.