Successful emergency endovascular aortic repair for intratumoral hemorrhage in extensive retroperitoneal mass of testicular origin.

BACKGROUND: Metastatic germ cell cancer of the testis is characterized by favorable prognosis since effective treatment methods are available even in cases of extensive disease. Retroperitoneal masses frequently encroach major blood vessels requiring a vascular intervention usually performed in association with the post-chemotherapy retroperitoneal lymph node dissection (RPLND). Reported clinical case describes a successful pre-treatment endovascular surgery for abdominal aortic rupture allowing for full-dose systemic chemotherapy administration, and subsequent radical surgical intervention at primary tumor site as well as metastatic retroperitoneal lymph node dissection including the reconstruction of inferior caval vein. CASE PRESENTATION: Patient presented with left-sided testicular tumor and voluminous retroperitoneal mass with vascular involvement. Soon after the patient had been admitted for the first cycle of cisplatin-based chemotherapy, computed tomographic angiography (CTA) revealed a dorsal aortic wall rupture with active extravasation and irregular pseudoaneurysmatic dilatation of the aorta below the leak area. Retroperitoneal intratumoral hemorrhage associated with the bilateral iliac venous thrombosis required an endovascular repair procedure of infrarenal abdominal aorta. CONCLUSIONS: Following the successful endovascular aortic repair 3 cycles of BEP (bleomycin, etoposide, cisplatin) regimen were administered with subsequent delayed left radical orchiectomy and RPLND associated with vena cava inferior (VCI) resection. Reconstruction of VCI was originally not deemed necessary as collateral blood flow appeared sufficient, however, intraoperative complications resulted in the need for unilateral VCI reconstruction, using the interposed bypass between right common iliac vein and infrarenal segment of VCI. Histopathologic examination of the attained specimen detected no vital cancer structures. The patient remains disease-free 18 months after the RPLND.

Clinical Management and Outcome in Extreme Retroperitoneal Growing Teratoma Syndrome of Testicular Origin - Clinical Management and Effect of the Treatment.

BACKGROUND: Growing teratoma syndrome (GTS) is an uncommon clinical finding in patients treated for testicular cancer. It is diagnosed during or after chemotherapy as an expanding tumour mass not responding to the treatment while the serum tumour markers are within the normal range. Pathological evaluation of resected tissue confirms the structures of benign mature teratoma. CASE: Authors report a case of metastatic germ cell testicular cancer treated with 2 lines of chemotherapy and everolimus, that had finally been subjected for the resection of voluminous metastatic masses. We give a brief overview of current records concerning clinical management of GTS, and support the major role of surgical treatment in GTS. RESULTS: Patient with metastatic mixed germ cell tumour of testis underwent a radical orchiectomy and completed the 1st line treatment with BEP (bleomycin, etoposide, cisplatin) regimen. Radiographic restaging showed considerable disease progression to the retroperitoneum and supraclavicular lymph nodes. Second-line treatment with VIP (etoposide, ifosfamide, cisplatin) did not reverse the progression and the patient was consulted at our institute. Following the enrolment to the clinical study with everolimus, the patient exhibited continual metastatic growth in contrast to serum markers decrease. GTS was confirmed after resection of enormous retroperitoneal tumour mass, as well as from the specimen obtained from the subsequent supraclavicular and hepatal metastasectomy. The patient attained complete remission and has been closely observed over the last 31 months since the last surgery. CONCLUSION: GTS is resistant to chemotherapy and radiation and complete surgical resection results in excellent disease control. Clinicians should be aware of this infrequent presentation of testicular tumours, to ensure the timely diagnosis and the appropriate surgical removal without any delay. Despite the great extent and vital vasculature encasement, surgery may be feasible and successful, as we report in our case, consistently with the published data.

Dia-gnostic Challenges and Extraordinary Treatment Response in Rare Malignant PEComa Tumor of the Kidney.

BACKGROUND: Epithelioid angiomyolipoma (EAML) of the kidney, in contrast to classic benign renal angiomyolipoma, is a rare mesenchymal neoplasm with malignant potential. Represent-ing a member of the perivascular epithelioid cells (PEComa) tumor family aris-ing from the perivascular epithelioid cells, its accurate dia-gnosis and therapeutic approach remains challenging. METHODS: We report a case of a patient with malignant EAML, initially treated as renal cell carcinoma (RCC) at our institution. In this paper, we briefly summarize current status of clinical and histopathological knowledge of renal PEComas with metastatic potential and reconsider the dia-gnostic and therapeutic approach in this particular case to highlight the risk of mis-dia-g----nosis, malignant potential of renal PEComas and to demonstrate an unexpected treatment response. RESULTS: The patient in our case was dia-gnosed with chromophobe RCC with sarcomatoid features. She underwent a radical nephrectomy and epinephrectomy with a satisfactory postoperative history. Local recurrence urged chemother-apy commencement with sunitinib in the first line, and shortly afterwards, the patient was enrolled in a clinical trial with everolimus, with an extraordinary favorable treatment response for 30 months. Follow-ing the extirpation of single abdominal nodularity after 36 months of treatment with mTOR inhibitor, and proceed-ing the everolimus administration, the dis-ease slowly progressed to the right liver lobe, result-ing in right hemihepatectomy in another 24 months. The immunoprofile of liver metastases with positive stain-ing of melanoma markers and smooth muscle markers induced the revaluation of the primary tumor and abdominal nodularity specimen to an invasive EAML of the kidney. Further dis-ease progression was unavoidable despite several chemother-apy regimens, and the patient died 104 months after primary dia-gnosis. CONCLUSIONS: Renal tumors with adverse radiographic and histopathological features should become candidates for immunohistochemical stain-ing as its omission frequently leads to a misdia-gnosis, as showed in our case report. Atypical treatment response might suggest a possibility of a diagnostic mistake and should lead to reevaluation of the diagnostic and treatment process in the particular patient. Key words: renal PEComa -  epithelioid angiomyolipoma -  dia-gnosis -  everolimus.

[Purtscher-like retinopathy as a complication of acute pancreatitis]. / Purtscher-like retinopatia ako komplikácia akútnej pankreatitídy.

INTRODUCTION: Purtscher-like retinopathy as a complication of acute pancreatitis is a disease which causes generalized retinal and macular oedema associated with retinal surface haemorrhage, which results in a permanent, sometimes partially reversible visual loss. CASE REPORT: The case report presented in this article refers to the case of a 36-year-old woman with severe acute pancreatitis. The pancreatitis led to the development of acute respiratory insufficiency with signs of septic shock and consequently, the patient was transferred to the critical care unit. The patient underwent a total of 8 surgical interventions with the revision of the abdominal cavity, necrectomy and application of the vacuum system into the abdominal cavity. Eye exams were conducted for impaired vision with the conclusion of Purtscher-like retinopathy associated with acute pancreatitis, occlusion of the central retinal artery of both eyes, confirmed by fluorescence angiography. After one year, alterations of the retina show no signs of improvement in this case; the loss of vision is permanent.Key words: acute pancreatitis Purtscher-like retinopathy.