Initial experience with laparoscopic posterior retroperitoneal adrenalectomy in single tertiary center

Purpose: Laparoscopic posterior retroperitoneal adrenalectomy (LPRA) is a surgical method that accesses the adrenal gland through the back. The aim of this study was to report initial experience of LPRA and evaluate possibilities for surgical application.Methods: From March 2018 to December 2019, a total of 30 consecutive patients diagnosed with adrenal tumor underwent surgical treatment at Pusan National University Hospital were enrolled. Clinicopathologic features and various peri- and postoperative parameters were analyzed by retrospective medical record review. The mean age of the patients was 48.20 ± 13.66 years.Results: The mean body mass index (BMI) was 25.50 ± 4.30 kg/m2 . Primary hyperaldosteronism was the most frequently preoperative diagnosed disease (n = 13, 43.4%), followed by adrenal incidentaloma (n = 8, 26.6%), Cushing syndrome (n = 5, 16.6%) and pheochromocytoma (n = 4, 13.3%). The mean size of postoperative adrenal tumor was 2.72 ± 1.76 cm. The mean operating time was 162 ± 58.14 minutes. Among the 30 patients, 28 patients underwent total adrenalectomy (93.3%) and two patients underwent cortical sparing adrenalectomy (6.7%). When LPRA was performed for patients with BMI > 23.16 kg/m2 , the operating time was longer than the average (P = 0.016).Conclusion: LPRA was suitable and safe for patients with benign adrenal tumors. BMI, retroperitoneal fat density and postoperative adrenal weight may be related to the operating time, so they should be considered when deciding on a surgical method for adrenalectomy.

Single-cell transcriptome analysis identifies a unique tumor cell type producing multiple hormones in ectopic ACTH and CRH secreting pheochromocytoma

Ectopic Cushing's syndrome due to ectopic ACTH&CRH-secreting by pheochromocytoma is extremely rare and can be fatal if not properly diagnosed. It remains unclear whether a unique cell type is responsible for multiple hormones secreting. In this work, we performed single-cell RNA sequencing to 3 different anatomic tumor tissues and 1 peritumoral tissue based on a rare case with ectopic ACTH&CRH-secreting pheochromocytoma. And in addition to that, 3 adrenal tumor specimens from common pheochromocytoma and adrenocortical adenomas were also involved in the comparison of tumor cellular heterogeneity. A total of 16 cell types in the tumor microenvironment were identified by unbiased cell clustering of single-cell transcriptomic profiles from all specimens. Notably, we identified a novel multi-functionally chromaffin-like cell type with high expression of both POMC (the precursor of ACTH) and CRH, called ACTH+&CRH+ pheochromocyte. We hypothesized that the molecular mechanism of the rare case harbor Cushing's syndrome is due to the identified novel tumor cell type, that is, the secretion of ACTH had a direct effect on the adrenal gland to produce cortisol, while the secretion of CRH can indirectly stimulate the secretion of ACTH from the anterior pituitary. Besides, a new potential marker (GAL) co-expressed with ACTH and CRH might be involved in the regulation of ACTH secretion. The immunohistochemistry results confirmed its multi-functionally chromaffin-like properties with positive staining for CRH, POMC, ACTH, GAL, TH and CgA. Our findings also proved to some extent the heterogeneity of endothelial and immune microenvironment in different adrenal tumor subtypes.

Non-syndromic pheochromocytoma: From post-operatory scores to lifetime follow-up

We introduce a case report of an adult male diagnosed with non-syndromic pheochromocytoma with a first pathological post-operatory report of malignant type with further re-considerations during follow-up for a 6-year period of time. This is 46 - year old male admitted for: post-adrenalectomy status reassessment. In 2013 he was diagnosed with high blood pressure requiring a complex regime of anti-hypertensive drugs to control it. In 2015 he was referred for an endocrine check-up which revealed a pheochromocytoma (noradrenaline type). Computed tomography imaging of the abdomen showed a right adrenal tumor of 28/38 mm, a mass that was clearly contoured, with heterogeneous pattern, and with moderate caption of intravenous contrast. Laparoscopic right adrenalectomy is performed with normalization of high pre-operatory normetanephrines and a dose reduction of anti-hypertensive medication which was still necessary. Pathological report suggested a malignant pheochromocytoma; the initial PASS score of 8 was later re-calculated, and a GAPP score of 5 was achieved showing a moderately differentiated tumor. No genetic backup was identified. Within the first year after tumor removal, the patient suffered a stroke, proving the higher cardiovascular risk than general population even after hormonal imbalance is restored. Lifelong surveillance is the rule.

Palliative Radiotherapy Provided Good Pain Relief for Painful Adrenal Metastasis

Adrenal metastases often occur in patients with metastasized lung cancer, but symptoms rarely develop. A 45-year-old man presented with right abdominal pain requiring strong opioids due to large right adrenal metastasis of lung adenocarcinoma. The tumor was 7.3 × 5.6 × 8.4 cm in size. He was treated with palliative radiotherapy (RT) up to 39 Gy in 13 fractions for this lesion without severe adverse effects. After RT, he had good pain relief, and opioids were no longer needed. Palliative RT for a large adrenal tumor can provide a good analgesic effect without relevant toxicity.

Gastric Leiomyoma Misdiagnosed as a Left Cystic Adrenal Tumor

Gastrointestinal leiomyomas often occur in the esophagus, colon, and rectum, but are very rare in the stomach. The patient was referred to Jeju national university medical center for the treatment of a left adrenal tumor diagnosed 5 years ago. She had a long history of repeated left flank pain, nausea, and abdominal distension for the last few years. Annual follow-up computed tomography scan showed a no changed 5×5.5-cm sized multiloculated left adrenal cystic lesion. Laboratory tests revealed that the basal levels of plasma adrenocorticotropin, cortisol, aldosterone, renin, testosterone, adrenaline, noradrenaline, and dopamine were normal findings. The preoperative diagnosis was a left nonfunctioning adrenal neoplasm with symptom of discomfort. However, the tumor revealed as an exophytic growing leiomyoma with cystic changes of a stomach by surgery. The mass was found at the posterior wall of the fundus and about 5-cm-sized mass. Resection of the tumor was performed using laparoscopy. The pathologic diagnosis revealed a gastrointestinal leiomyoma with a benign cystic lesion lined by respiratory epithelium with an old hemorrhage and chronic gastritis. The patient had no evidence of recurrence during the 12-month follow-up. Gastric leiomyoma is unfamiliar to the usual urologists. It needs to take gastric leiomyoma into account if the left adrenal tumor is close to the stomach. Herein, we report the case of a 44-year-old woman with gastrointestinal leiomyoma misdiagnosing as a nonfunctioning adrenal tumor.