International Journal of Research and Scientific Innovation (IJRSI) |Volume IX, Issue IX, September 2022|ISSN 2321-2705
A Huge 5kg-Intra-Abdominal Ancient Schwannoma with A Recurrence-A Case Report.
Michael Abiodun ADEYANJU
Department of Surgery, Federal Medical Centre, Ebute-Metta, Lagos. Nigeria.
Abstract: Introduction- This is a rare case of a recurrent huge intrabdominal Ancient Schwannoma, the size of which has not been reported before. This case report aims to enrich the literature on cases of undiagnosed abdominal masses.
Clinical features-The patient complained of huge abdominal distension, weight loss, and breathlessness of 4 months duration.
The important clinical findings were multiple cutaneous painless swellings, hyperpigmented patches on the trunk, and a mass occupying the whole of the abdomen.
Management-A diagnosis of visceral manifestation of neurofibromatosis was made to rule out an intra-abdominal malignancy. Preoperative Abdominal Computed Tomography (CT) scan revealed a huge intrabdominal mass while a CT-guided biopsy showed a Benign Mesenchymal tumor. He had an exploratory laparotomy, and the mass was totally excised. The recovery was uneventful, and the outcome was excellent. Histopathology showed that the tumor was a Schwannoma with no malignancy. However, 5 months after, the swelling recurred and he was eventually lost to follow up.
Conclusion- The rarity of Intra-abdominal Schwannomas and absent or non-specific signs and symptoms present the surgeon with a diagnostic dilemma. Preoperative Abdominopelvic CT scan, Ultrasound scan, and Magnetic Resonance Imaging are necessary imaging modalities. Complete excision at surgery reduces recurrence. Close follow-up is essential.
Keywords- Schwannoma, Neurofibromatosis, Café-au-lait
I. INTRODUCTION
Ancient Schwannomas are benign masses that rarely occur in the abdomen and even when they occur, they rarely get as big as was seen in this case. They occur more as rare solitary1 nerve sheath tumors of peri-neural Schwann cells, that affect the nerves of the extremities, trunk, and the head and neck region. They are commoner in females2, between the third and fifth decades, occur spontaneously but could also present as part of Carney’s complex, (comprising conjunctival disorders, myxomas, schwannomas, and other endocrine disorders like Cushing’s syndrome)3, or in association with a group of genetic autosomal dominant disorders called neurofibromatosis 1 and 2 (NF1 & NF2)4. They grow slowly5 in the abdomen without signs and symptoms until they occupy the available space and then begin to impinge on surrounding structures to cause intermittent pain. They could be benign or malignant in the abdomen, or sarcoma in the retroperitoneal space. Only 0.5-5% of all retroperitoneal tumors are Schwannomas5. Malignant transformation is very rare.