Background: Melanotic schwannoma (MS) is a rare variant of peripheral nerve sheath tumor. MS commonly arises along the spinal nerve sheath. Patients most often experience pain along the dermatome of the affected nerve root. Symptoms development is usually insidious. About half of MS cases are associated with Carney complex, a multi-neoplastic disorder. The remaining cases arise spontaneously. About 10–44% of these tumors undergo malignant transformation. Case Description: We describe a case of hemorrhagic MS presenting as acute chest pain mimicking myocardial infarction, a presentation which has not yet been described in the literature. Neurologic examination did not reveal any abnormalities. Myocardial infarction was ruled out in the ER, and a chest CT angiogram was ordered for evaluation of PE or aortic dissection which revealed an intradural extramedullary dumbbell-shaped mass extending through the left vertebral foramen at the level of T8. MRI revealed a heterogenous mass that was hyperintense with T2 and hypointense with T1-weighted imaging. The patient underwent an open laminectomy of the left T8 and T9 vertebrae and gross total resection (GTR) of a hemorrhagic black tumor. Microscopic examination showed fascicles and nests of plump spindle cells with variable intracellular melanin. Immunohistochemistry showed the cells to be positive for S100, SOX10, HMB-45, and MART-1, confirming diagnosis of MS. Two months after the operation, the patient was doing well and is free of recurrence. Conclusion: GTR is considered the optimal treatment for MS; radiotherapy and chemotherapy may be considered but have not been shown to improve patient outcomes.
Henoch-Schönlein purpura (HSP) is a self-limited vasculitis that affects children and the preadolescent population. It is characterized by the deposition of immunoglobulin A immune complexes in tissues leading to palpable purpura, abdominal pain, arthritis, and nephropathy. When it occurs in adults, the clinical manifestations are the same; however, adults present with more significant renal involvement. While abdominal pain is the most common gastrointestinal (GI) manifestation, it can also present with GI bleeding, intussusception, bowel ischemia, and bowel perforation. Here, we report the case of a 22-year-old gentleman who presented with nonspecific GI complaints such as nausea, vomiting, and loose stools. He was later found to have terminal ileitis preceding the onset of rash, the biopsy of which confirmed HSP. Terminal ileitis is a rare GI manifestation of HSP and is not very commonly reported in the literature.
Platypnea-orthodeoxia syndrome (POS) is defined by dyspnea and deoxygenation due to a change in body position from lying down to an upright position. We present a case of a large right atrial (RA) thrombus likely due to a right coronary artery fistula in a patient with a patent foramen ovale (PFO). On imaging, the thrombus was thought to be an atrial myxoma involving the tricuspid valve; however, after surgical excision and histopathological analysis, it was noted to be a cystic thrombus. Red-brown material along with vascular elements was noted on histopathology. Post-surgery, the patient was critically ill and died due to severe tricuspid regurgitation (TR) and hypotension despite using a right ventricle assist device and multiple vasopressors. Reverse Lutembacher syndrome (RLS) is defined as a triad of tricuspid stenosis (TS), elevated RA pressure, and right-to-left atrial shunting. The location of the mass and positional changes could be causing transient RLS from positional TS and interatrial shunting via the PFO causing POS. Cardiac magnetic resonance imaging can help differentiate between intracardiac masses. T1 and T2 signal characteristics and differences in contrast enhancement can help differentiate between a thrombus and a tumor. Treatment options include anticoagulation, thrombolysis, and thrombectomy. If severe TR occurs after surgery, treatment modalities such as caval valves could be an option in the future. Extracorporeal membrane oxygenation to provide right ventricle support in such cases could be considered.
Solitary fibrous tumours (SFTs) are rare mesenchymal tumours that are mostly seen in the pleura. Lately, they have also been described in other locations. Recent discovery of the NAB2-STAT6 fusion gene which is specific for SFTs has led to an accurate diagnosis of SFTs. The occurrence of SFTs in the mesentery is very rarely reported in the literature. We report a case of a 63-year-old female who presented with abdominal pain, rectal bleeding and Fusobacterium bacteraemia, who was ultimately found to have a mesenteric SFT.
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