Retroperitoneal Fibrosis Is Still an Underdiagnosed Entity with Poor Prognosis.

BACKGROUND: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles abdominal organs including the aorta and ureters. Data on the incidence of this disease are limited. SUMMARY: The disease may be idiopathic or secondary to infections, malignancies, drugs, or radiotherapy. The idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in the aorta and surrounding tissues. Taking into account the dominant symptoms and clinical characteristics of patients with periaortitis, 2 subtypes of disease could be distinguished. The vascular subtype includes patients with nondilated aorta or with inflammatory abdominal aortic aneurysm, both with and without involvement of adjacent structures and with numerous risk factors for atherosclerosis. In the renoureteral subtype, obstructive uropathy manifesting with hydronephrosis and acute kidney injury is the predominant finding. Due to the variety of symptoms, diagnosis of RPF remains challenging, difficult, and often delayed. A series of diagnostic tests should be performed, in order to confirm the diagnosis idiopathic RPF. Laboratory workup includes evaluation of inflammatory indices and immunological studies. A biopsy and histopathological evaluation may be necessary to confirm diagnosis and differentiate the disease. Computed tomography, magnetic resonance imaging, and positron emission tomography are the modalities of choice for the diagnosis and follow-up of this disease. Management of ureteral obstruction, hydronephrosis, and aortic aneurysms often requires surgical evaluation and treatment. The pharmacological treatment of RPF has been evaluated in a few randomized trials and is mainly based on observational studies. Steroid therapy remains the gold standard of treatment. KEY MESSAGES: Nowadays, multidisciplinary team approach with clinical and diagnostic experience in both primary and secondary RPF as well as 2 major subtypes should be offered. Centers specialized in rare diseases with collaboration with other units and referral system yield the best possible outcomes.

Retroperitoneal fibrosis, a rare entity with urorenal and vascular subtypes - preliminary data.

INTRODUCTION: Retroperitoneal fibrosis (RPF) is a rare disease associated with the formation of hard inflammatory and fibrous tissue in the retroperitoneum. Taking into consideration the fact that RPF is a rare disease with different subtypes, we compared the basal clinical and biochemical characteristics of the vascular and urorenal subtypes. PATIENTS AND METHODS: From January 2005 until December 2021, 27 patients were identified as vascular subtype (18 males) and 11 as urorenal subtype (9 males). RESULTS: Patients with a primary urorenal origin had significantly worse kidney function as reflected by serum creatinine and eGFR (both p < 0.001); they also had higher serum cholesterol (p < 0.01). Hypertension, diabetes, hyperlipidemia and nicotinism were significantly more prevalent in vascular subtype (all p < 0.001). CONCLUSION: Vascular subtype is more prevalent in our study with more cardiovascular risk factor present. Due to the diversity of symptoms, diagnosis of RPF becomes a challenge for specialists as well as therapy.

Tandem peptide receptor radionuclide therapy using 90Y/177Lu-DOTATATE for neuroendocrine tumors efficacy and side-effects - polish multicenter experience.

INTRODUCTION: One of the concepts of theranostics in nuclear medicine is peptide receptor radionuclide therapy (PRRT), whereby labeled somatostatin analogs are used for imaging and treating inoperable or disseminated neuroendocrine tumors (NET). AIM: The aim of the study was to determine the therapeutic efficacy and toxicity of tandem 90Y /177Lu-DOTATATE in patients with disseminated NET in a multicenter trial. MATERIALS AND METHODS: 103 patients with NET G1/G2 treated with 90Y/177Lu-DOTATATE (1:1) with amino-acid infusion for nephroprotection were included in the study. RESULTS: Overall survival from the disease diagnosis (OS-D) was 127.4 months and from the time of PRRT (OS-T) was 89.5 months. Progression-free survival (PFS) was 29.9 months. An analysis based on the proliferation index revealed a statistically significant impact on PFS and OS-T (PFS G1 vs G2, 59.3 vs 24.3 months; OS-T G1 vs G2, not reached vs 79.9 months). The effect of the primary disease site was also analyzed. For pancreatic vs small bowel vs large bowel, the PFS was 30.8 vs 30.3 vs 40.6 months, the OS-T was 94 vs 61.9 vs 131.2 months and OS-D was 130.4 vs 89.2 vs not reached months, respectively. The 2-year risk of progression was 42%. The probability of 2-year and 5-year overall survival was 89% and 62%, respectively. PRRT was well tolerated by all patients. One patient (1%) developed myelodysplastic syndrome. No other grade 3 and 4 hematological or renal toxicity was observed. CONCLUSIONS: This multicenter trial showed that tandem 90Y/177Lu-DOTATATE is highly effective and safe therapy for patients with disseminated NET.

Familial SDHB gene mutation in disseminated non-hypoxia-related malignant paraganglioma treated with [90Y]Y/[177Lu]Lu- DOTATATE.

Familial paraganglioma may be related to mutations in succinate dehydrogenase (SDH) enzyme complex genes. Among patients with hereditary paraganglioma, SDH subunit B (SDHB) gene mutations are associated with the highest morbidity and mortality related to a higher malignancy rate. We report a family with the c.689G>A (p.Arg230His) mutation in the SDHB gene identified in two family members, a father and his daughter. While the 14-year-old daughter had no evidence of clinical disease, recurrent and later disseminated [131I]metaiodobenzylguanidine uptake-negative head and neck paraganglioma with multiple bone metastases developed in the father who underwent peptide receptor radionuclide therapy with [90Y]Y/[177Lu]Lu-dodecane tetraacetic acid octreotate (DOTATATE) at the time of the genetic diagnosis. This treatment was repeated 6 years later due to disease progression and the patient, who is currently 49 years old, remains alive and in good overall clinical condition at 8 years of follow-up after the original presentation at our unit. The growing armamentarium of imaging methods available for such patients may inform decision making regarding choice of the optimal treatment approach, potentially contributing to improved outcomes.

Atrial fibrillation does not affect ankle-brachial index measured using the Doppler method.

Atrial fibrillation may affect blood pressure measurements. The ankle-brachial index (ABI) is a ratio of systolic blood pressure measured on the lower and upper limbs that may also be affected by arrhythmia. The purpose of the study was to investigate whether atrial fibrillation influenced ABI results. Ninety-nine patients (age 66.6±11 years, 63 males and 36 females) who underwent electrical cardioversion of atrial fibrillation were investigated. ABI measurements using the Doppler method were performed on both lower extremities before and after electrical cardioversion. Measurements were repeated three times and then averaged. The ABI using both lower limbs was lower before electrical cardioversion than after restoration to sinus rhythm (right side: 1.132 (1.065-1.210) during atrial fibrillation vs. 1.179 (1.080-1.242) in sinus rhythm, P=0.019; left side: 1.142 (1.075-1.222) during atrial fibrillation vs. 1.170 (1.098-1.255) in sinus rhythm, P=0.011). However, the upper 95% confidence interval (CI) margins for the median differences in ABI were 0.045 and 0.040 for right and left, respectively, suggesting that the observed difference was clinically insignificant. There was a significant correlation between measurements obtained before and after electrical cardioversion on both lower limbs (r=0.61, P<0.001 and r=0.67, P<0.001). The Bland-Altman plot showed good agreement between measurements performed using the Doppler method during atrial fibrillation and sinus rhythm. Study results showed that atrial fibrillation did not have a clinically important effect on ABI measurements.

TeleNEN as a telemedicine model for neuroendocrine neoplasm management in case of Meckel's diverticulum NET.

A case of 25- years-old female with NET deriving from Meckel's diverticulum is described. The patient had one year history of dermatological skin problems. Ultrasound examination of abdomen performed because of arterial hypertension, revealed multiple hepatic lesions, which was confirmed in contrast enhanced CT. The typical contrast enhanced metastatic lesions in CT and elevated levels of chromogranin A suggested NET of unknown origin. SRS with 99mTc-HYNICTOC was perform for primary tumor localization, and revealed liver and paraaortic lymph nodes metastases, but no sign of primary tumor location. As a next step for primary tumor localization 68Ga-DOTATATE PET/CT was done, which revealed focus of increased uptake in small intestine considered to be the primary tumor site. The imaging and clinical history of patient was discussed on ENETS Tumor Board. Due to location of primary tumor in the small intestine with no anatomical changes in CT, laparotomy guided with gamma probe after 68Ga-DOTATATE injection was performed. During surgery procedure, the primary tumor was hardly palpable in the tip of Meckel's diverticulum, confirmed by gamma probe. After surgery, tandem peptide receptor radionuclide therapy (PRRT) was started. Patient received 4 doses of 90Y/177Lu-DOTATATE with total activity of 360 mCi (13.32 GBq). The three months follow up 68Ga-DOTATATE PET/CT had shown stable disease of patient. The presented case showed importance role of multidisciplinary team cooperation in patient management. Use of RGS is essential in cases like presented, when the tumor cannot be localized only by surgical palpation.

The local spread of pheochromocytoma after adrenalectomy with a rupture of the tumor capsule at the time of the surgery.

INTRODUCTION: We present a case of a 29-year-old patient treated due to fully symptomatic pheochromocytoma of the right adrenal gland. CASE PRESENTATION: Patient was operated on and an open right-sided adrenalectomy was performed. At the time of the surgery, a rupture of the tumor capsule occurred. Five years post-operatively, a recurrence of the symptoms of chromaffin-cell tumor was noted. After the exact localization of the multiple recurrences, the patient was reoperated on. CONCLUSION: The case of pheochromocytoma is presented due to the possibility of chromaffin-cell seeding into the peritoneum, with no signs of distal metastases so far.

Laparoscopic treatment of adrenal cysts--own research and literature review.

MATERIAL AND METHODS: Over the last 18 months we operated on six patients with large adrenal gland cysts in our centre. This consisted of 8.2% of all patients treated in said period due to adrenal gland pathologies. On ruling out malignancy or parasitic nature of the lesions, all patients were surgically treated in order to excise the cysts while leaving the gland untouched. In five patients the cysts were resected but the adrenal gland was spared. However, in one patient the adrenal gland coated the entire cystic mass, which imposed performance of adrenalectomy in addition to cystectomy. During surgeries we tried not to clip the suprarenal vein, which we managed to do in four out of six cases. RESULTS: A one-year remote follow-up period revealed no cyst recurrence in ultrasound or CT, and it was possible to visualise the remaining part of the adrenal gland in all cases. CONCLUSION: Thus, in our opinion resection of benign cysts is well justified and recommendable.

A spontaneous paraganglioma-pheochromocytoma syndrome.

We present a case of a 40-year old woman diagnosed with a four-place spontaneous paraganglioma-pheochromocytoma syndrome, which was treated surgically. The presence of the succinate dehydrogenase complex subunit D (SDHD) mutation that causes the pheochromocytoma was confirmed but no mutations in the family members were found. After the excision of the paragangliomas located in the areas of the division of carotid arteries, and mediastinum, as well as a tumor on the left site of the celiac trunk, the patient remains asymptomatic and is regularly followed-up.

Adrenal tumors - diagnostics and the factual situation.

The study presented three cases of patients diagnosed with adrenal tumors subject to surgical intervention during the past 6 months in our Department. The patients presented with radiological diagnostic difficulties, as to the character and location of the primary tumor. The aim of the study was to demonstrate differences between radiological examination results and the factual situation observed during the adrenalectomy. In all the presented cases patients' were subject to laparoscopic intervention. In two cases conversion to open surgery was necessary. The histopathological results of the surgically removed samples were as follows: leiomyoma, myoperycytoma and pheochromocytoma. In selected cases imaging examinations might be of limited value, especially when determining the character and location of the primary lesion of the adrenal gland.

[Heart failure following lumbar surgery]. / Niewydolnosc serca u chorej po operacyjnym leczeniu dyskopatii w odcinku ledzwiowym kregoslupa.

High-output heart failure may be caused by an arteriovenous fistula. A case is reported of an arteriovenous fistula following discectomy.