Acta medica Lituanica

A Message from the New Editor-in-Chief

Rimantas Jankauskas — Sat, 13 Jun 2020 00:00:00 +0300

Improvement in childhood cancer survival in Lithuania over three decades

Jelena Rascon, Giedrė Smailytė — Sat, 13 Jun 2020 00:00:00 +0300

Background. Population-based EUROCARE-5 studies demonstrated that childhood cancer survival rates in Lithuania were 10–20% lower than the European mean. We aimed to analyse the change in the outcome of treatment of paediatric malignancies in Lithuania over 30 years. Methods. A single-centre retrospective analysis of children below 18 years of age treated for cancer at Vilnius University Hospital Santaros Klinikos between 1982 and 2011 was carried out. The minimal requirement of 5-year follow-up after diagnosis was specified for survival estimation. The vital status was assessed using data from the population-based Lithuanian Cancer Registry. To evaluate changes over time, the entire cohort was split into three groups according to the time of diagnosis: 1982–1991, 1992–2001, and 2002–2011. Results. A total of 1268 children met the inclusion criteria. The shortest median follow-up was 8.9 (IQR 6.4–11.5) years for patients treated in the third decade. The 5-year overall survival of the entire cohort increased from 37.3% (95% CI 30.2–44.3) in 1982–1991 to 70.7% (95% CI 66.4–74.1) in 2002–2011 (p < 0.0001). The same trend was evident when calculated separately for leukaemia (p < 0.0001), lymphoma (p < 0.0005), and solid tumours (p < 0.004). The percentage of cure rose from zero in the early years of the period analysed to 80% in 2010 and 2011. The improvement in the treatment outcome was attributable to the reduction of treatment-related mortality from 45.8% in 1982–1991 to 12.4% in 2002–2011 and disease recurrence from 30.4% to 19.6% for the same periods, respectively. Conclusions. Significant progress in the cure rate of children treated for cancer at our institution was observed over 30 years. Collaborative national and international clinical and research efforts are crucial to ensure further advances in care and cure.

Initial experience with Edwards SAPIEN valve transcatheter implantation in native RVOT in Latvia

Sat, 13 Jun 2020 00:00:00 +0300

Transcatheter pulmonary valve implantation has been a well-known method for more than a decade, but there are still many challenging cases when a personalized solution is needed. We report a case of a 15-year-old female patient with tetralogy of Fallot, who underwent a surgical correction during infancy. Because of progressive pulmonary regurgitation, stenosis, and right ventricle dilatation, transcatheter pulmonary valve implantation in the native right ventricle outflow tract (RVOT) using Edwards SAPIEN valve was performed. A “landing zone” was created prior to the intervention of stenting the RVOT and the right pulmonary artery. The transcatheter approach for pulmonary valve replacement in a native RVOT is a reasonable alternative to the surgical approach.

High-grade well-differentiated neuroendocrine tumour of the cecum diagnosed following incisional hernia repair: a case report

Sat, 13 Jun 2020 00:00:00 +0300

High-grade well-differentiated neuroendocrine tumour tumours (NETs) of gastrointestinal tract are rare; they can arise in any part of the digestive system and usually present in advanced stages. Low incidence and wide heterogeneity in the biological behaviour of such lesions pose a diagnostic and therapeutic challenge. Loco-regional NETs should be resected whenever feasible, potentially with a curative intent. Management of a metastatic disease is often more complex, primarily aimed at the alleviation of symptoms, prevention of further complications, and prolongation of survival. Current literature describing the optimal treatment plan for such patients is sparse and further studies are necessary to enhance our understanding of the disease. Here we present a case of high-grade well-differentiated cecal NET with an associated carcinoid syndrome that was diagnosed following the incisional hernia repair.

Von Hippel-Lindau syndrome and renal tumours: radiological diagnostic and treatment options. A case report and literature review

Audrius Untanas, Mantas Trakymas, Indrė Lekienė, Rūta Briedienė — Sat, 13 Jun 2020 00:00:00 +0300

Background. Von Hippel-Lindau disease (VHL) is a rare autosomal dominant syndrome diagnosed for 1 out of 36000–45000 newborns and 90% of the patients have a clinical manifestation before 65 years of age. Affected individuals have an increased risk of developing tumours in several organs or their systems. The most common tumours are retinal or central nervous system hemangioblastomas (60–80%) and VHL-associated renal lesions. Contrast-enhanced computer tomography (CECT) is the gold standard for the diagnosis and characterization of renal tumours. The best treatment option for VHL syndrome-caused renal tumours are nephron-sparing treatment techniques (cryotherapy, radiofrequency, or microwave ablation), which require imaging control. All these innovative treatment techniques are extremely important for VHL patients, because they increase the quality of life by staving off renal dialysis and preventing distant metastases. Case report. Our case report presents a 16-year-old female with multiple renal cysts observed on ultrasound examination and clinically and molecularly diagnosed with Von Hippel-Lindau syndrome (deletion of the entire VHL gene). After that, for past 11 years multiple renal tumours were removed by cryoablation and patient monitoring on contrast-enhanced magnetic resonance (MRI) and CECT control scans was conducted. Conclusions. Active multidisciplinary patient follow-up, routine radiological examinations, and correct treatment tactics allow controlling the progression of renal cell carcinoma and other tumours associated with VHL syndrome, maintaining a normal organ function for a long time, and preventing distant metastases and fatal disease outcomes.

Partial nephrectomy can be a successful treatment option for renal epithelioid angiomyolipoma: a case report and literature review

Sat, 13 Jun 2020 00:00:00 +0300

Background. Renal epithelioid angiomyolipoma is a rare tumour which involves kidneys in most cases. It is known for its aggressive behaviour as a significant number of cases have been associated with metastatic epithelioid angiomyolipoma. Usually, radical treatment with systemic therapy is recommended. Only a small number of cases of epithelioid angiomyolipomas have been reported with the standard treatment being radical or partial nephrectomy. We present a case report showing that partial nephrectomy can be a successful treatment option for renal epithelioid angiomyolipoma. This is the first case of this nature in Lithuania. Case presentation. In this case, a 40-year-old male with epithelioid angiomyolipoma of the left kidney is presented. In 2012, a cystic left renal mass 40 × 41 mm in size was diagnosed incidentally while performing ultrasound and later confirmed by MRI. Due to the size of the tumour and the possibility of renal cell carcinoma, surgery was scheduled. Left partial nephrectomy was performed successfully. Final pathology report came back with the diagnosis of renal epithelioid angiomyolipoma. The patient had yearly follow-up for six years by CT scan, and neither recurrence nor progression were observed. Conclusion. Early detection and diagnosis are crucial for treatment as the tumour tends to have malignancy potential. With early diagnosis, partial nephrectomy can be performed with yearly follow-up and no systemic treatment required.

Challenges in the diagnostics and management of hydrochlorothiazide-induced severe hyponatremia in a habitual beer drinker

Gabija Laubner, Gabija Gintautaitė, Robertas Badaras — Sat, 13 Jun 2020 00:00:00 +0300

Introduction. Beer potomania is a syndrome of severe hyponatremia caused by excessive beer consumption. The risk of hyponatremia increases in the case of a combination of beer potomania and the use of thiazide diuretics. Case report. A 55-year-old male patient with the anamnesis of a long-lasting alcohol use disorder was presented to the emergency department after seizures accompanied by an impaired mental status. He had been drinking beer regularly for ten years. On physical examination, the patient was tachypneic, tachycardic, disorientated, restless, the Glasgow Coma Scale score of 9, observed tremor, and the smell of alcohol from the mouth. Laboratory results showed plasma sodium 105 mmol/L, blood urea nitrogen 1.8 mmol/L, the alcohol concentration in the blood 0.06 g/l, and calculated serum osmolality 219 mOsm/kg H₂O. After a detailed initial evaluation of the patient and labs for hyponatremia, a diagnosis of beer potomania was established. On the third day of hospitalization, the patient’s anamnesis was filled with information about the use of Valsartan/hydrochlorothiazide, together with reduced salt intake in the diet for three months for arterial hypertension treatment. It was decided that the combination of heavy beer drinking with the use of diuretic and reduced consumption of salt provoked hyponatremia. The patient was treated with infusion therapy of sodium chloride; plasma sodium level reached 136 on the third day of treatment. Conclusions. This case represents an unusual syndrome of beer potomania in conjunction with hydrochlorothiazide usage and reduced salt consumption, which can result in severe hyponatremia that may provoke severe neurologic damage.