Haemorrhagic retroperitoneal paraganglioma initially manifesting as acute abdomen: a rare case report and literature review

Abstract

Background Paragangliomas (PGLs) are extremely rare neuroendocrine tumours arising from extra-adrenal chromaffin cells. PGLs are clinically rare, difficult to diagnose and usually require surgical intervention. PGLs mostly present catecholamine-related symptoms. We report a case of Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal PGL. There has been only one similar case reported in literature. Case presentation We present a unique case of a 52-year-old female with acute abdomen induced by haemorrhagic retroperitoneal PGL. The patient had a 5-h history of sudden onset of serve right lower quadrant abdominal pain radiating to the right flank and right lumbar region. Patient had classic symptoms of acute abdomen. Abdominal ultrasound revealed a large abdominal mass with a clear boundary. A Computed Tomography Angiography (CTA) of superior mesenteric artery was also performed to in the emergency department. The CTA demonstrated a large retroperitoneal mass measured 9.0 × 7.3 cm with higher density inside. A provisional diagnosis of retroperitoneal tumour with haemorrhage was made. The patient received intravenous fluids, broad-spectrum antibiotics and somatostatin. On the 3rd day of admission, her abdominal pain was slightly relieved, but haemoglobin decreased from 10.9 to 9.4 g/dL in 12 h suggesting that there might be active bleeding in the abdominal cavity. Thus, we performed a midline laparotomy for the patient. Haemorrhage was successfully stopped during operation. The retroperitoneal tumour with haemorrhage was completely removed. The abdominal pain was significantly relieved after surgery. The patient initially presented with acute abdomen instead of catecholamine-related symptoms. The diagnosis of retroperitoneal PGL with haemorrhage was finally confirmed by postoperative pathological and immunohistochemical results. The postoperative course was uneventful. At the 1-year follow-up visit, no tumour recurrence was observed by Single Photon Emission Computed Tomography. A literature review was performed to further understand and analyse the aforementioned disease. Conclusion Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal paraganglioma is extremely rare. Abdominal Computed Tomography is essential to locate the lesion and differentiate between other causes of acute abdomen. PGLs are hypervascular tumours. We should be aware that ruptured retroperitoneal PGL with massive bleeding could be life threatening and require emergency laparotomy.

Spontaneous Retroperitoneal Bleeding as a Complication of Unusual Renal Paraganglioma

“…Spontaneous rupture of nonfunctioning retroperitoneal paraganglioma is rare and quite difficult to be diagnosed initially as it presents with severe abdominal pain that mimics other acute surgical conditions [ 39 , 40 ]. Our patient was also asymptomatic until the acute abdomen discomfort caused by hemorrhagic retroperitoneal paraganglioma.…”

A rare case of retroperitoneal paraganglioma located in the neck of the pancreas: a case report and literature review

“…PGL deriving from the thorax, abdomen and pelvic regions overproduces some hormones, especially norepinephrine, but PGL derived from the head and neck region is generally biochemically silent (5). PGL was mainly presented to be local compression symptoms and some of them produce catecholamine which causes hypertension.…”

A patient with paraganglioma undergoing laparoscopic resection: A case report

“…Sympathetic PGLs, originating from the chest and abdominal sympathetic nerves, account for 80% of all PGLs 2 . Moreover, 85% of sympathetic PGLs occur beneath the diaphragm and are particularly observed in the retroperitoneum around the adrenal and renal areas around the organ of Zuckerkandl and the bladder 3,4 .…”

“…Cytological characteristics include granular cytoplasm, prominent nucleoli, vesicular nuclei, pseudoinclusions inside nuclei, and so on. There may be secondary changes like hemorrhage, hemosiderin deposition, sclerosis, and pigmentation of the lipofuscin or melani 2,36 …”