A 67-year-old man was introduced to examine for abnormal findings in physical examination. Upper gastrointestinal endoscopy detected the 3 lesions and diagnosed multiple gastric cancers with biopsy. Laparoscopic total gastrectomy was performed and final pathological diagnosis was pT2N1M0, pStage ⅡA with HER2 positive(3+). Adjuvant chemotherapy was not administrated. CT findings 6 months after procedure showed 2 liver metastases(S6, S7)and laparoscopic partial liver resection was performed. Pathological findings proved the metastasis of previous gastric cancer. Although adjuvant chemotherapy of S-1 was started 2 months after hepatectomy, CT findings showed second recurrences in liver and right adrenal gland. Chemotherapy was changed to S-1 plus cisplatin(SP)plus trastuzumab. AS these recurrence sites and ascites could not be detected by CT after 6 courses, therapeutic effect was judged as clinical CR(cCR)and SP plus trastuzumab was stopped due to side effect. Since then, regular follow-up CT showed preservation of status of cCR without chemotherapy and he has been alive without recurrence for 8 years and 9 months after gastrectomy. We report a case of recurrent gastric cancer of multiple organ metastasis with long-term survival due to multimodal treatment.

Patients with central nervous system (CNS) involvement at initial diagnosis of extranodal NK/T-cell lymphoma (ENKTL) are exceedingly rare, and the clinicopathologic features of CNS involvement have not been well characterized. In this study, we reviewed 662 patients with ENKTL from August 2008 to September 2019. Their clinical and pathological features, treatments, and survival outcomes were analyzed. The median follow-up time was 72 months. Nine of 662 (1.4%) patients were diagnosed with CNS involvement. Among them, the median age was 37 years, and seven patients were male. All patients had positive EBV-DNA, and three patients were asymptomatic at the time of diagnosis with CNS involvement. Common extranodal involved sites included bone, paranasal sinuses, breast, kidney, adrenal gland, and bone marrow. All patients were positive for cytoplasmic CD3ε, cytotoxic granule proteins, and EBER and negative for CD20. All patients received intrathecal chemotherapy and at least one cycle of systemic chemotherapy. Seven patients had died and two were still alive by the last follow-up. The median overall survival (OS) in patients with CNS involvement at initial diagnosis of ENKTL was 9 months, and the 1-year OS was 44.4%. Five patients achieved a complete response after asparaginase-based chemotherapy; two were still alive, one died of systemic progression, one died of ENKTL-associated hemophagocytic syndrome, and one died of treatment-related infections. In conclusion, CNS involvement at initial diagnosis of ENKTL is extremely rare with poor prognosis. There is no standard treatment, and asparaginase-based chemotherapy combined with intrathecal chemotherapy might yield good efficacy.

25-60% of septic patients experience relative adrenal insufficiency (RAI) and glucocorticoid (GC) is frequently used in septic patients. However, the efficacy of GC therapy and whether GC therapy should be based on the status of RAI are highly controversial. Our poor understanding about the pathogenesis of RAI and a lack of RAI animal model present significant barriers to address these critical issues.

Psychiatrist manifestations-especially acute psychotic episode-of primary hyperaldosteronism is not very common in the scientific literature. The main symptoms are high blood pressure and hypokalemia, the diagnosis seems to be based on the determination of aldosterone and plasma renin activity, also the treatment includes majorly adrenalectomy. The take-away lesson from this case is to think of organic cause in front of acute psychotic episode.

Adequate oxygen levels are essential for the functioning and maintenance of biological processes in virtually every cell, albeit based on specific need. Thus, any change in oxygen pressure leads to modulated activation of the hypoxia pathway, which affects numerous physiological and pathological processes, including hematopoiesis, inflammation, and tumor development. The Hypoxia Inducible Factors (HIFs) are essential transcription factors and the driving force of the hypoxia pathway; whereas, their inhibitors, HIF prolyl hydroxylase domain (PHDs) proteins are the true oxygen sensors that critically regulate this response. Recently, we and others have described the central role of the PHD/HIF axis in various compartments of the adrenal gland and its potential influence in associated tumors, including pheochromocytomas and paragangliomas. Here, we provide an overview of the most recent findings on the hypoxia signaling pathway in vivo, including its role in the endocrine system, especially in adrenal tumors.

Neuroblastoma (NB) is an undifferentiated malignant tumor of the sympathetic ganglia, occurring in children under 5 years of age. However, it is a rare histology in adult patients, occurring once per every 10 million patients per year. We present the case of a 68-year-old male patient presented to our department for right lumbar pain, asthenia, loss of weight and altered general status. The contrast-enhanced abdominal computer tomography revealed bilateral adrenal tumoral masses of 149 mm and 82 mm on the right and left sides, respectively, with invasion of the surrounding organs. The patient underwent right 3D laparoscopic adrenalectomy and right radical nephrectomy. The pathological result concluded that the excised tumor was a neuroblastoma of the adrenal gland. The patient followed adjuvant oncological treatment; however, due to disease progression, he passed away 22 months after the surgery. To our knowledge, less than 100 cases of adrenal NB in adult patients have been published, the eldest case being diagnosed at 75 years of age; meanwhile, the largest reported tumor measured 200 mm, and was excised through open surgery. Minimally invasive techniques have been limited so far to smaller, organ-confined diseases, thus making the present case the largest adrenal NB removed entirely laparoscopically. Neuroblastoma in the adult population is a rare finding, with worse prognosis compared to pediatric patients. The available literature does not provide enough data for standardized, multimodal management, as the patients are treated following adapted pediatric protocols, thus reinforcing the need for international, multidisciplinary boards for rare tumors.

Glucocorticoids (GCs) are anti-inflammatory and immunosuppressive steroid molecules secreted by the adrenal gland and regulated by the hypothalamic-pituitary-adrenal (HPA) axis. GCs present a circadian release pattern under normal conditions; they increase their release under stress conditions. Their mechanism of action can be via the receptor-independent or receptor-dependent pathway. The receptor-dependent pathway translocates to the nucleus, where the ligand-receptor complex binds to specific sequences in the DNA to modulate the transcription of specific genes. The glucocorticoid receptor (GR) and its endogenous ligand cortisol (CORT) in humans, and corticosterone in rodents or its exogenous ligand, dexamethasone (DEX), have been extensively studied in breast cancer. Its clinical utility in oncology has mainly focused on using DEX as an antiemetic to prevent chemotherapy-induced nausea and vomiting. In this review, we compile the results reported in the literature in recent years, highlighting current trends and unresolved controversies in this field. Specifically, in breast cancer, GR is considered a marker of poor prognosis, and a therapeutic target for the triple-negative breast cancer (TNBC) subtype, and efforts are being made to develop better GR antagonists with fewer side effects. It is necessary to know the type of breast cancer to differentiate the treatment for estrogen receptor (ER)-positive, ER-negative, and TNBC, to implement therapies that include the use of GCs.

The case of a 45-year-old male patient diagnosed with European Network for the Study of Adrenal Tumours (ENSAT) criteria, stage IV adrenocortical carcinoma (ACC) with unexpectedly prolonged survival is being reported. The patient underwent resection of stage IV ACC and despite suboptimal adherence to postoperative mitotane and chemotherapy, had a prolonged survival spanning almost seven years. The possible reasons for such an outcome are discussed. ACC is a rare tumour with stage 4 disease known to be associated with a particularly grim prognosis. A low grade on histology (mitotic index 11-12 per 50 HPF) was likely responsible for the prolonged survival of our patient. Low grade disease may predict extended survival in stage IV ACC.

Lung cancer is the leading cause of cancer-related death worldwide, with frequent metastases to the brain, liver, adrenal glands, and bone. The incidence of intraluminal small bowel metastases of the lung is extremely rare and poorly documented within the literature. Few case studies have been published since the late 1980s and early 1990s. However, little is known about this rare form of metastasis. Small bowel metastatic disease has atypical symptoms that mimic a variety of other diseases; as a result, signs and symptoms may be overlooked until the disease has progressed to a late stage. Signs of small bowel obstruction, symptomatic anemia, abdominal pain, and peritonitis are commonly reported signs and symptoms. Various modalities can be utilized for the workup of suspected small bowel metastasis, including positron emission tomography, computed tomography, and various forms of endoscopy. The prognosis for lung cancer patients with intestinal metastases is poor, with many only surviving months to a few years after diagnosis. Therefore, it is critical to consider small bowel masses as a differential diagnosis in a patient with primary lung cancer who demonstrates clinical signs consistent with symptomatic anemia secondary to gastrointestinal (GI) bleeding, peritonitis, or small bowel obstruction. We report an unusual case of intraluminal and fungating small bowel masses in a patient who had previously undergone lung resections and chemo-immunotherapy. She was diagnosed with non-small undifferentiated carcinoma with tumor necrosis over 12 years before disease recurrence in the bilateral lungs, right adrenal gland, bone, and small bowel. The discovery of the small bowel metastases occurred while undergoing treatment for advanced-stage disease. At this time, she completed chemo-immunotherapy and remained on maintenance immunotherapy. The patient also underwent a partial right adrenalectomy and radiotherapy to the right adrenal gland. Given that she was experiencing symptomatic anemia and further workup indicated that the GI masses were causing her anemia, she underwent palliative small bowel resection of the masses. The pathology results demonstrated that the masses originated from her primary lung cancer, confirming metastatic disease to the small bowel.

Space-occupying lesions of the adrenal glands are one of the most frequent tumors; however, only a fraction of approximately 20% need further diagnostics and treatment. The diagnostic standard is native computed tomography (CT). For larger tumors and those that cannot be clearly classified as benign, the supplementary radiological modalities magnetic resonance imaging (MRI), contrast CT and 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) should be used. Adrenal gland neoplasms can be hormone-active or hormone-inactive. The most important hormone-active adrenal gland neoplasms are those with autonomous cortisol secretion and autonomous aldosterone secretion as well as pheochromocytomas. Several laboratory tests are available each for the respective hormone activity. The indications for surgery are dependent on the dignity, size and hormone activity. Minimally invasive surgical techniques have become more important due to advantages such as lower blood loss; nevertheless, open surgery is still indispensable for large suspected tumors. Care must be taken preoperatively and postoperatively, particularly in cases of hormone-active tumors.

Background: Idiopathic granulomatous mastitis (IGM) is a chronic inflammatory condition of the mammary gland that presents as a painful mass, and it must be distinguished from both infectious mastitis and breast cancer. When diagnosed during lactation, it can result in significant distress and early weaning. Injection of triamcinolone has been used as a successful treatment method, but safety in breastfed infants has not been established. Methods: We present a case of a lactating patient who received a direct injection of triamcinolone (dosage 40 mg) in her breast to treat IGM after failure of oral corticosteroids. Breastmilk samples were expressed by the patient 0, 1, 4, and 24 hours after the procedure, and then daily for 1 week. All the samples were analyzed using liquid chromatography mass spectrometry. The patient was supported by a breastfeeding and lactation medicine clinic. Results: After injection of triamcinolone into the granulomatous mass, breast milk samples were collected and analyzed. No samples were found to contain triamcinolone. A temporary but significant decrease in milk production was noted after injection, though only a slight decrease had been noted with 6 weeks of systemic corticosteroids. With support, the patient rebuilt milk production and continued to breastfeed from both breasts. Conclusion: Triamcinolone was not found in any milk samples (≥0.78 ng/mL) following therapeutic injection of the affected breast. The patient was able to continue breastfeeding from the affected breast with intermittent symptoms.

Diagnosis of pheochromocytoma or paraganglioma (PPGL) in pregnancy has been associated historically with high rates of materno-fetal morbidity and mortality. Recent evidence suggests outcomes are improved by recognition of PPGL before or during pregnancy and appropriate medical management with alpha-blockade. Whether antepartum surgery (before the third trimester) is required remains controversial and open to case-based merits. Women with PPGL in pregnancy are more commonly delivered by Caesarean section, although vaginal delivery appears to be safe in selected cases. At least some PPGLs express the luteinizing hormone/chorionic gonadotropin receptor (LHCGR) which may explain their dramatic manifestation in pregnancy. PPGLs in pregnancy are often associated with heritable syndromes, and genetic counselling and testing should be offered routinely in this setting. Since optimal outcomes are only achieved by early recognition of PPGL in (or ideally before) pregnancy, it is incumbent for clinicians to be aware of this diagnosis in a pregnant woman with hypertension occurring before 20 weeks' gestation, and acute and/or refractory hypertension particularly if paroxysmal and accompanied by sweating, palpitations and/or headaches. All women with a past history of PPGL and/or heritable PPGL syndrome should be carefully assessed for the presence of residual or recurrent disease before considering pregnancy.

We report a case of long-term survival in paraganglioma treated with repetitive surgery. A 32-year-old man was seen at the hospital because of hypertension and headache. On biochemical tests, elevated serum and urinary levels of noradrenaline were noted. Abdominal computed tomographic (CT) scan revealed a tumor 50 mm in diameter on the dorsal region of the inferior vena cava, superior to the renal artery. Our diagnosis was paraganglioma and we performed open resection of the tumor. Six years later, hypertension and headache appeared and abdominal computed tomography revealed tumors located in right renal hilus and para-aortic regions superior to aortic bifurcation, and ¹²³I-MIBG scintigraphy revealed uptake at the same sites. Therefore, we diagnosed the patient with recurrent pheochromocytoma and performed laparoscopic resection of the tumor. Surgical treatment was repeatedly performed for recurrence, and the symptoms due to hypersecretion of catecholamines could be controlled over a long period.

BACKGROUND Adrenocortical carcinoma (ACC) is a rare malignancy associated with unfavorable prognosis. It is mainly diagnosed in the fifth or sixth decade of life. Symptoms of ACC are associated with hormonal activity, presence of metastases, and size of the tumor. The treatment and prognosis depend on the stage of the disease assessed with the ENSAT staging system. CASE REPORT A 38-year-old White man was admitted to our department from the city hospital due to a huge hematoma of the right adrenal gland (130×100 mm). On admission, the patient's condition was stable, and no active bleeding or other complications were present. Therefore, initially, conservative treatment was performed. The control CT scan showed reduction of the hematoma (90×80 mm). Due to the unknown character of the tumor and the sudden onset of bleeding, the patient was prepared for elective surgery according to the phaeochromocytoma surgery protocol. Following preparation, the patient underwent right-sided adrenalectomy. In the postoperative histopathological examination, adrenocortical carcinoma was diagnosed, which allowed the patient to receive appropriate oncological treatment. CONCLUSIONS There is currently no clear algorithm for the management of adrenal hemorrhage. A hemodynamically unstable patient requires urgent surgical treatment. Patients in good general condition should be prepared for early elective surgery.

Stereotactic MR-guided Radiotherapy (MRgRT) is an interesting treatment option for adrenal gland metastases (AGM). We reviewed data from 12 consecutive patients treated with MRgRT for an AGM in our center between 14 November 2019 and 17 August 2021. Endpoints were tolerance assessment, the impact of adaptive treatment on target volume coverage and organs at risk (OAR) sparing, local control (LC), and overall survival (OS). The majority of patients were oligometastatic (58.3%), with 6 right AGM, 5 left AGM and 1 left and right AGM. The prescribed dose was 35 to 50 Gy in 3 to 5 fractions. The median PTV V95% on the initial plan was 95.74%. The median V95% of the PTVoptimized (PTVopt) on the initial plan was 95.26%. Thirty-eight (69%) fractions were adapted. The PTV coverage was significantly improved for adapted plans compared to predicted plans (median PTV V95% increased from 89.85% to 91.17%, p = 0.0478). The plan adaptation also significantly reduced Dmax for the stomach and small intestine. The treatment was well tolerated with no grade > 2 toxicities. With a median follow-up of 15.5 months, the 1-year LC and OS rate were 100% and 91.7%. Six patients (50%) presented a metastatic progression, and one patient (8.3%) died of metastatic evolution during the follow-up. Adaptation of the treatment plan improved the overall dosimetric quality of MRI-guided radiotherapy. A longer follow-up is required to assess late toxicities and clinical results.