Bilateral Familial Pheochromocytoma: Difficulty of Diagnosis and Management

Introduction: Bilateral pheochromocytoma is a rare tumor, often seen in the context of a family illness. The majority of pheochromocytomas are sporadic, but they can also occur within the framework of genetic diseases (10%): MEN2, VPL, NF1, familial paraganglioma (mutation of SDHB)⋯ In the context of a familial genetic disease, the pheochromocytoma can be either unilateral or bilateral, benign in 95% of cases and malignant in 5%. It still poses several problems related to its diagnosis, genetic aspects, especially in the absence of a family history. And the criteria of malignancy, given the non-existence to date of a certain & universal criterion that judges the malignancy of the tumor. We present through this article the exceptional case of “bilateral familial pheochromocytomas with strong suspicion of malignancy”. Clinical Case: A 29-year-old lady, hypertensive since 4 years, admitted for exploration of severe secondary hypertension. She reports a triad of menard and severe lumbar pain, the somatic examination shows hypersensitivity of the flanks, a BP of 240/120 mmhg. the abdominal CT scan confirmed by an MRI show a right adrenal mass of 5 cm & another left of 1 cm, of suspicious appearance (irregular contours, areas of necrosis, heterogeneity, spontaneous density at 35 HU, a wash out at 33% with presence of L4 spinal angioma and peri-aortic lymph nodes. urinary methoxylated derivatives (UMD) returned high. The genetic study was positive VPL (on various radiological tests, there was just a spinal angioma) We retained the diagnosis of familal bilateral pheochromocytoma. In front of the clinical & radiological signs of malignancy, we decided to do: a right total adrenalectomy & a left partial adrenalectomy. Unfortunately, the blood pressure didn’t drop, and UMD were still positive; the anatomopathological study shows a pheochromocytoma with a PASS score estimated at 3 (benign!) Faced with conflicting clinical-radiological and histological data, we decided to total adrenalectomy without lymph node dissection and to closely monitor the progress. the post Op blood pressure was normalized and the UMD returned negative. Conclusion: Malignant familial bilateral pheochromocytoma is a very rare & very difficult entity to diagnose, manage & monitor. A good management requires serious collaboration between: endocrinologist, radiologist, urologist, pathologist

Wearing Surgical Masks Coupled With Restricting the Flow of People in Patient Wards Versus Preventive Atomization Inhalation in Preventing Fever After General Anesthesia Surgery: A Retrospective Analysis During COVID-19

Background: The COVID-19 could be transmitted through aerosols, and aerosol can be produced by atomization inhalation. Preventative aerosol inhalation is prohibited in our hospital during COVID-19, however the number of cases of fever after surgery has not increased significantly. We want to know whether wearing surgical masks coupled with restricting the flow of people in patient wards has same effect with preventive atomization inhalation in preventing fever after surgery, and we wonder whether preventive atomization inhalation is unnecessary during COVID-19, as long as strictly wearing surgical masks and restricting the flow of people in patient wards have been met.Methods: Eight kinds of common surgery were covered in this retrospective analysis, including total thyroidectomy (for the treatment of thyroid carcinoma), total adrenalectomy (adrenal tumor), radical gastrectomy (gastric cancer), radical nephrectomy (renal cell carcinoma), radical prostatectomy (prostate cancer), radical resection for sigmoid colon cancer, radical resection for rectal cancer and appendectomy (appendicitis). Cases in Group A underwent preventive atomization inhalation whilst cases in group B wore surgical masks and restricted the flow of people in patient wards. Occurrence of fever, occurrence of fever recurrence and the maximum temperature in the first week after surgery were analyzed in this study.Results: No significant differences can be seen between group A and group B in terms of occurrence of fever, occurrence of fever recurrence and the maximum temperature after surgery in the first week.Conclusion: Wearing surgical masks combined with restricting the flow of people in patient wards has same effect with preventive atomization inhalation in preventing fever after general anesthesia surgery, which means, during COVID-19, preventive atomization inhalation might not be necessary as long as strictly wearing surgical masks and restricting the flow of people in patient wards have been met.

SPECTRUM OF SOMATIC MUTATIONS IN RETROPERITONEAL LEIOMYOSARCOMA: CLINICAL CASE AND LITERATURE REVIEW

Retroperitoneal leiomyosarcomas (RpLMS) are highly aggressive tumors, which are characterized by poor prognosis and resistance to chemotherapy. Targeting tumor-specific molecular pathways have become a rapidly expanding field in drug development to increase efficacy of treatment of LMS. Here we present a case report of rapidly progressive RpLMS with gene mutations of key molecular pathways, which have not previously described in the literature. A 61-year-old man was admitted to our hospital with complaints of abdominal pain and fever. Radiological examination revealed retroperitoneal leiomyosarcoma, which was histologically confirmed by core-biopsy. The patient underwent radical (R0) en-bloc resection of tumor with left hemicolectomy, left total nephrectomy, left total adrenalectomy and distal subtotal pancreatectomy. Pathological assessment of the tumor revealed G3 leiomyosarcoma. The patient did not receive adjuvant therapy. Disease progression (local recurrence and pulmonary metastases) occurred 3 months after surgery, and the patient died 6 months after surgery. Immunohistochemical study revealed positive PD -L1 expression in tumor cells. The percentage of PD -L1- expressing cells was 30 %. Molecular-genetic testing allowed identification of somatic mutations in genes, such as PIK3CA, ALK, EGFR, ERBB, ESR1 and PD GFRA and confirmation of microsatellite stable status (MSS) of the tumor. Further studies to investigate spectrum of mutations in RpLMS are of great interest, since they can allow identification of potential targets for more effective antitumor therapy and to improve treatment results.

A New Insight into the Surgical Treatment of Primary Macronodular Adrenal Hyperplasia

Purpose This prospective study presents the results of a new approach in the treatment of primary macronodular adrenal hyperplasia (PMAH), with simultaneous total adrenalectomy of the larger adrenal gland and partial adrenalectomy of the contralateral adrenal gland (adrenal-sparing surgery). Materials and Methods We performed a prospective study including 17 patients with PMAH treated surgically with adrenal-sparing surgery in a tertiary referral hospital, with a median follow-up of 41 months. Clinical, hormonal, and genetic parameters were evaluated before surgery and during follow-up. All patients had at least 1 radiological examination before and after the procedure. Results Among the 17 patients, all but 1 patient had complete hypercortisolism control, and 12 recovered normal adrenal function after surgery. Significant improvement in clinical parameters was observed: weight loss (P = .004); reduction of both systolic (P = .001) and diastolic (P = .001) blood pressure; and reduction in the number of antihypertensive drugs (P < .001). Intra-, peri-, and postoperative complications were not observed. Conclusion Adrenal-sparing surgery is a safe and feasible procedure to treat patients with PMAH, providing a substantial chance of hypercortisolism control without the disadvantages of lifetime corticosteroid replacement.