Challenges in the diagnosis and treatment of extrauterine leiomyomas: case series

Extrauterine leiomyoma is a rare disease with multiple potential metastasizing sites. Pathogenesis of this condition remains debatable with previous myomectomy a considerable risk factor. Diagnosis and treatment remain a challenge due to diverse presenting symptoms and the initial involvement of various specialties, which often cause distress in these patients. In this article, we present one case of pulmonary leiomyomas on expectant management, one case of wide-disseminated extrauterine leiomyomas including the bones, spines, soft tissues and peritoneum who underwent decompression laminectomy and biopsy of paravertebral mass and one case of retroperitoneal leiomyoma who underwent resection of the lesion.

Lymphoma presented as dysphagia: a diagnosis hard to swallow

An 18-year-old woman presented with progressive oesophageal dysphagia, weight loss and night sweats over a 6-month period. Oesophagogastroduodenoscopy revealed a diffuse luminal narrowing with normal mucosa, whose biopsies were inconclusive. A cervical and thoracic CT scan showed a thickening of the upper oesophagus, densification of the mediastinal fat, several adenopathies and a 4.3×2.4 cm mass with infiltrative appearance and heterogeneous enhancement in right cervical paravertebral location. Positron emission tomography-CT showed marked increased fluorodeoxyglucose uptake in supradiaphragmatic lymph nodes, pleuropulmonary tissue, paraspinal musculature and bone marrow. Imaging-guided and surgical incisional biopsies of the paravertebral mass were inconclusive. During hospitalisation, she developed right cervicobrachial paraesthesia. Only excisional biopsy of the mass allowed the diagnosis of high-grade B-cell lymphoma not otherwise specified, Ann Arbor stage IV-B. The patient underwent chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone), followed by R-EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin hydrochloride). Follow-up at 12 months revealed complete response.

Perioperative control of paroxysmal hypertension using esmolol with alpha-blockade in a child with a germline mutated paraganglioma

Summary The use of antihypertensive medications in patients with pheochromocytomas and paragangliomas (PCC/PG) is usually a challenge. We report a case of familial paraganglioma that was successfully treated by esmolol and other antihypertensive medications without associated perioperative complications. Our patient was an 11-year-old girl who presented with classic symptoms and signs of PCC/PG and a CT scan of the abdomen that showed a right-sided paravertebral mass. Her father was diagnosed with paraganglioma a few years ago. Prazosin had been started but she continued to experience uncontrolled paroxysms of blood pressure (BP). She was known to have asthma; hence, she developed serious bronchospasm with atenolol. She was, therefore, switched to esmolol that successfully controlled her BP in addition to prazosin and intermittent doses of hydralazine prior to laparoscopic surgery with no side effects of medications or postoperative complications. Esmolol could be a good alternative to routinely used beta-blockers in children with PCC/PG with labile hypertension and related symptoms in the pre and intra-operative periods. It is titrable, effective, and can be weaned rapidly helping to avoid postoperative complications. Further larger studies on the use of esmolol in children with PCC/PG are needed to confirm our observation. Learning points In addition to alpha-blockers, esmolol could be a good alternative for routinely used beta-blockers to control paroxysmal hypertension and tachycardia in the pre- and intra-operative periods. Esmolol is titrable and an effective beta-blocker. It can be weaned rapidly helping to avoid postoperative complications in children with PCC/PG. Children with PCC/PG and other comorbidity like asthma may particularly benefit from the use of esmolol due to no or less side effects on airway resistance and the advantage of rapid titration of the medication compared to other beta-blockers.

Paraganglioma in the posterior mediastinum: a case report

Background Paragangliomas are tumors that arise from extra-adrenal chromaffin cells. Herein, we present a rare case of a functional paraganglioma in the posterior mediastinum. Case presentation A 36-year-old man presented with paroxysms of chest pain and headache. At presentation, the patient’s blood pressure was 190/120 mmHg. Chest computed tomography and magnetic resonance imaging revealed a left paravertebral mass in the posterior mediastinum. 123I-metaiodobenzylguanidine scanning revealed focally high tracer uptake in the left paravertebral area. The metanephrine level in the urine was elevated, confirming a rare, catecholamine-producing, functional paraganglioma in the posterior mediastinum. Before surgery, the patient was prepared by orally administering α- and β-adrenergic blockers. The mass was then resected via a lateral thoracotomy. The metanephrine level in urine was normal 24 h after surgery. Conclusions Paragangliomas in the posterior mediastinum are very rare, but more than half of all cases are functional. The associated symptoms are curable with complete resection, and long-term follow-up for recurrence is important.

Case of functioning thoracic paraganglioma

Functioning thoracic paraganglioma (PGL) is rare in clinical practice. We present a 33-year-old man with this pathology, who came with right-sided chest pain and was found to have a right-sided paravertebral mass. Fine needle aspiration cytology revealed a PGL. Urine normetanephrine was elevated and meta- iodobenzylguanidine scan showed increased tracer uptake in the right hemithorax, suggestive of a functioning neuroendocrine tumour. The patient was subjected to right PGL excision by video-assisted thoracoscopic surgery, after adequate preoperative preparations. The perioperative period was uneventful, except for a transient rise in blood pressure during the surgery. His blood pressure continued to be normal in the postoperative period. In any patient with a paravertebral mass, the possibility of PGL should be kept in mind even if the patient is normotensive. Making a preoperative diagnosis is important, because excision of functioning PGL without adequate preoperative preparation may be detrimental.