Morphological Characterization of Papillary Renal Cell Carcinoma Type 1, the Efficiency of Its Surgical Treatment.

AIM: Papillary renal cell carcinoma type 1 (pRCC1) represents the second most common type of malignant renal epithelial tumour. The origin of its characteristic appearance, its growth mechanism, and the long-term efficiency of its surgical treatment remain uncertain. Our aim was to determine typical characteristics of surgically treated pRCC1. METHODS: pRCC1 was verified in 83 of 1,629 (5.1%) kidney tumours surgically treated in the period of January 2007-January 2016. The clinical and radiological characteristics, type of surgery, histopathology results and follow up data were recorded. Spearman correlation, Kruskal-Wallis analysis of variance, Fisher's exact, and chi-square test were used to analyse appropriate variables. The overall survival rate was evaluated using the Gehan-Wilcoxon test and the Cox proportional hazards model. RESULTS: The mean tumour size was 52.0 mm (15-180); 98.8% of the tumours showed a spherical shape and in 82.1%, exophytic growth was observed. Partial nephrectomy was performed in 80.7%. A majority (81.9%) were classified as pT1. Tumours, 89.2% of them, belonged to Fuhrman grade 1 or 2. The mean follow-up was 46.8 months. The overall survival was associated with pT category (p ≤ 0.0001). CONCLUSIONS: Typical signs of pRCC1 are a spherical shape, exophytic growth and low Fuhrman's grade. More than three-fourths of pRCC1 could be treated by the nephron-sparing surgery.

Carney complex: a clinicopathologic and molecular biological study of a sporadic case, including extracutaneous and cutaneous lesions and a novel mutation of the PRKAR1A gene.

BACKGROUND: Carney complex (CNC) is an autosomal dominant disorder associated with multiple neoplasms. OBJECTIVE: We report a case of a 40-year-old Caucasian man with a sporadic form of CNC. METHODS: This is a clinicopathologic description and molecular biological study with an emphasis on histopathologic findings. RESULTS: The patient presented with multiple cutaneous myxomas, cardiac myxomas, and spotty pigmentation at typical sites. Additionally, a blue nevus, a lipoma, multiple calcifications in both testes, and hypoechogenic areas suspected of being adenomas in the thyroid gland were found. Microscopically, the 2 cardiac and 6 cutaneous myxomas studied manifested a typical appearance, being composed of scattered polygonal, stellate, plump and/or spindle cells in a mucinous matrix containing small, sometimes dilated blood vessels. Of the 6 cutaneous myxomas, only in one lesion was there an abnormal epithelial component (tiny basaloid buds and a horn cyst). Molecular biologic study revealed a heterozygous shift mutation c.796dupA in exon 10 of the PRKAR1A gene. Physical examination and genetic testing of family members (both parents and two brothers) for the PRKAR1A mutation were negative, as was analysis of the peripheral blood of 110 randomly selected, unrelated healthy individuals for the above mutation. These findings suggest sporadic disease and a novel mutation in our patient. LIMITATIONS: None. CONCLUSION: Herein we report a case of sporadic CNC in which a novel mutation in PRKAR1A was identified.

Portal and mesenteric vein thromboses in a patient with prothrombin G20210 mutation, elevated lipoprotein (a), and high factor VIII.

A 65-year-old man was examined for abdominal pain. Portal and mesenteric vein thromboses were described by ultrasound and computed tomography. No local cause was found. The patient had a positive history of venous thromboembolism. Thrombophilia workup revealed prothrombin G20210A mutation (heterozygous), C677T mutation of methylenetetrahydrofolate reductase gene (homozygous), elevated level of lipoprotein (a), and high level of coagulation factor VIII. Anticoagulation was started and planned for a long-term duration. The etiology of portal vein thrombosis is often multifactorial, with various combinations of systemic factors (inherited or acquired prothrombotic conditions) and local precipitating factors (inflammation, injury to the portal venous system, cancer of the abdominal organs, cirrhosis). The reported prevalence of hypercoagulable states in patients with portal vein thrombosis has been very heterogeneous so far. Some authors support a role of the prothrombin G20210A mutation. In the reported patient, this mutation was revealed in a combination with other hypercoagulable states.

The Prevalence of Concomitant Deep Vein Thrombosis, Symptomatic or Asymptomatic, Proximal or Distal, in Patients With Symptomatic Pulmonary Embolism.

Patients with pulmonary embolism (PE) may have symptomatic or asymptomatic concomitant deep vein thrombosis (DVT). The reported prevalence of PE-associated DVT is variable, and thus, the utility of routine testing is controversial. The aim of our study was to analyze the prevalence of DVT and the factors associated with proximal DVT/whole-leg DVT in patients with symptomatic PE. In 428 consecutive patients (mean age: 59 ± 16.4 years; 52.3% men), we performed clinical examination and complete bilateral compression ultrasound and ascertained medical history and risk factors for DVT/PE. χ2 and t tests were used. Deep vein thrombosis was found in 70.6%; proximal DVT in 49.5%. Sensitivity/specificity of DVT symptoms was 42.7%/93.7% for whole-leg DVT and 47.6%/83.3% for proximal DVT. Male gender significantly prevailed among those with whole-leg DVT and with proximal DVT (58.9% and 61.8%). Active malignancy was significantly more frequent in the patients with proximal DVT than without proximal DVT (10.4% vs 3.7%). In conclusion, the prevalence of PE-associated DVT is quite high but clinical diagnosis is unreliable. In our group, male gender and active malignancy were significantly associated with the presence of concomitant proximal DVT.

Extensively cystic renal neoplasms in adults (Bosniak classification II or III)--possible "common" histological diagnoses: multilocular cystic renal cell carcinoma, cystic nephroma, and mixed epithelial and stromal tumor of the kidney.

OBJECTIVE(S): To give an algorithm for resolution of extensively cystic renal neoplasms, preoperatively classified in the Bosniak classification as a category II and III. METHODS: From 1991 to 6/2004, 701 patients with 727 renal tumours were surgically treated at our hospital. Extensively cystic tumours were found in 10 cases. Extensively cystic tumours were defined as multicystic tumours without any solid nodules visible neither on CT, nor grossly in the specimen at operation (the Bosniak classification type II or III). RESULTS: Seven multilocular cystic renal cell carcinomas, three mixed epithelial and stromal tumour of the kidney and one cystic nephroma were diagnosed on histology. CONCLUSION(S): Extensively cystic renal tumours classified as the Bosniak type II or III correspond histologically to the entities mentioned above (multilocular cystic renal cell carcinoma, cystic nephroma, mixed epithelial and stromal tumour of the kidney). These entities cannot be distinguished one from another on preoperative imaging studies. A preoperative biopsy and intra-operative frozen-section analysis do not lead to a correct diagnosis in many cases. Fortunately, the operative strategy is the same for all these tumours. In such cases, the nephron sparing surgery is indicated, whenever technically feasible, as almost all extensively cystic renal tumours have a good prognosis.

Laparoscopic urinary bladder diverticulectomy combined with photoselective vaporisation of the prostate.

INTRODUCTION: Pseudodiverticulum of the urinary bladder is mostly a complication of subvesical obstruction (SO). The gold standard of treatment was open diverticulectomy with adenectomy. A more contemporary resolution is endoscopic, in two steps: the first transurethral resection of the prostate (TURP), the second laparoscopic diverticulectomy (LD). AIM: To present a one-session procedure - photoselective vaporisation of the prostate (PVP) with LD. MATERIAL AND METHODS: From 1/2011 to 6/2014, 14 LDs were performed: 1 LD only, 1 with laparoscopic radical prostatectomy, 12 combined with treatment of benign prostatic hyperplasia (BPH), 4 cases of TURP and LD in the second period. In 8 cases, PVP and LD in one session were combined. These 8 cases are presented. 3D CT cystography was used as a gold standard for assessment of diverticulum. RESULTS: The mean age was 66.5 ±5.5 (57.3-75.1) years, the mean size of the diverticulum 61.8 ±22.1 (26-90) mm. The procedure starts in the lithotomy position. It includes PVP and stenting of the ureter(s). Changing of position and laparoscopy follows: four ports, transperitoneal extravesical approach. Photoselective vaporisation of the prostate was performed using the Green Light Laser HPS (1x) or XPS with cooled fibre MoXy (7x). The mean delivered energy in PVP was 205.1 ±106.4 (120-458) kJ. The mean time of operation was 165.0 ±48.5 (90-255) min. No postoperative complications were observed. One patient underwent TUR incision after 1 year for sclerosis of the bladder neck. CONCLUSIONS: Pseudodiverticulum of the urinary bladder (with or without SO) is a relatively rare disease. One session of PVP (Green Light Laser XPS, MoXy fibre) and laparoscopic (transperitoneal extravesical) diverticulectomy is the preferred method for treatment of subvesical obstruction due to BPH and bladder diverticulum at our institution.

MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)].

INTRODUCTION: MiT translocation renal cell carcinomas (TRCC) predominantly occur in younger patients with only 25% of patients being over 40 years. TRCC contains two main subgroups with translocations involving 6p21 or Xp11.2. Herein we present 10 cases. MATERIALS: Eight cases were treated at main author's institution (identified among 1653 (0.48%) cases of kidney tumours in adults). Two cases were retrieved from the Pilsen (CZ) Tumour Registry. RESULTS: Six cases were type Xp11.2 and four 6p21; 7 female, 3 male patients; Xp11.2 4:2, 6p21 3:1. The mean age 49 years (range: 21-80), 5 patients (50%) over 40 years. The mean age of the group with Xp11.2 TRCCs was 55 (median 51) and 6p21 41 (32) years. One female with a 6p21 tumour (24 years) underwent nephrectomy at 4 months of pregnancy. Stage (UICC, 7th ed. 2009) was 5xI, 3xIII, 2xIV. The mean size of tumour was 80 (40-165) mm. The mean follow-up was 33.2 (1-92) months. In patients with 6p21 tumours, one (25%) died after 3 months due to widely metastatic disease. In patients with Xp11.2 tumours, 3 (50%) succumbed due to metastatic disease (range 1-8 months). Three patients with Xp11.2 are alive at 7, 52 and 92 months of follow-up, were diagnosed at early stage (T1a). CONCLUSION: TRCCs were more common in females. Patient with 6p21 tumours were younger than those with Xp11.2. Both types have definitive malignant potential Type Xp11.2 seems to be a more aggressive neoplasm than 6p21. The case with metastatic 6p21 tumour is the 4th case described in the English literature.

Laparoscopic adrenalectomy for metachronous ipsilateral metastasis following nephrectomy for renal cell carcinoma.

INTRODUCTION: Although laparoscopic adrenalectomy (LA) is considered as a gold standard approach for adrenalectomy, there are minimal data describing options and outcomes of LA after previous ipsilateral nephrectomy (PIN). AIM: To describe our results in a group of patients who underwent LA after PIN. MATERIAL AND METHODS: From August 2004 to October 2012 we performed at our institution 88 LA. Of this amount we performed 5 LA for metachronous metastasis of renal cell carcinoma (RCC) after PIN. This group was compared to a group without previous nephrectomy. RESULTS: The group comprised 4 men (80%) and 1 woman (20%); the mean age at the time of surgery was 66.8 ±8.5 (range: 60-77) years; the mean period between nephrectomy and adrenalectomy was 5.2 (range: 1.5-14) years; the operating time was longer in patients after PIN for 7 min; the mean blood loss was higher by 22 ml; duration of hospitalization was shorter by 1.3 days, paradoxically, compared with patients without PIN. There was no need for conversion to open surgery and we did not observe any other complications. CONCLUSIONS: Laparoscopic adrenalectomy for metastasis of RCC after PIN is a technically feasible method in selected patients and it is associated with no significant differences in perioperative data in comparison with the group without prior nephrectomy. The patients benefit from minimally invasive surgery. The performance has required an experienced laparoscopic surgeon.

Angiomatoid change in polyps of the nasal and paranasal regions: an underrecognized and commonly misdiagnosed lesion--report of 45 cases.

We present 45 patients with angiomatoid polyps of the nasal and paranasal regions (APNPRs), which are underrecognized lesions which may cause considerable diagnostic difficulties. There were 32 men and 13 women in our series. The average age at diagnosis was 49 years in men and 54.3 years in women. Locations were known in 41 cases and included the nasal septum (14), maxillary sinus (12), ethmoid sinuses (5), lateral wall of the nasal cavity (5), sphenoid sinus (1), and nasal cavity, not otherwise specified (4). X-ray or computed tomography was performed in 19 cases and revealed bone erosions/deviations in four cases. Initial misdiagnoses submitted by referring pathologists were reported in 20/32 of the consultation cases. Our study confirms that APNPRs are benign lesions which often recur and sometimes multiple recurrences are seen. APNPRs sometimes cause severe changes of the skeletal bones especially in recurrent lesions. Awareness of the above described features and familiarity with the clinical presentation of APNPRs is the best way to avoid a misdiagnosis.

Prostatic-type tissue in the lower female genital tract: a morphologic spectrum, including vaginal tubulosquamous polyp, adenomyomatous hyperplasia of paraurethral Skene glands (female prostate), and ectopic lesion in the vulva.

The investigators report a series of prostatic-type lesions occurring in the lower female genital tract. The cases included a 4.5-cm mass representing hyperplasia of the glandular and stromal tissue of paraurethral Skene gland, a small ectopic prostatic lesion in the vulva, and 4 tubulosquamous vaginal polyps. All lesions were immunopositive for prostate-specific antigen and/or prostatic acid phosphatase. A brief discussion of the earlier published material is included.

Rupture of papillary renal cell carcinoma.

OBJECTIVE: Typical signs of papillary renal cell carcinoma (PRCC) are extensive necroses of the tumorous mass, which can modify the clinical appearance of PRCC. These necroses can imitate cysts on radiological examinations (ultrasonography and CT). The tumours are fragile and vulnerable to spontaneous rupture or rupture following minimal trauma (i.e. they act as a locus minoris resistentiae). MATERIAL AND METHODS: A total of 650 patients with a total of 671 renal tumours were surgically treated at our hospital between January 1991 and December 2003. RESULTS: In 16 cases bilateral tumours were found (in all cases RCC) and in five cases two types of tumour were identified in one kidney [all were a combination of conventional RCC (CRCC) and PRCC]. Altogether, 621 tumours (92.5%) were diagnosed as RCCs. Of these, CRCC was found in 563 cases (90.7%), PRCC in 36 (5.8%), chromophobe RCC in 14 (2.3%) and unclassified RCC in 7 (1.1%). All cases of ruptured PRCC were included in our study. Interestingly, only PRCCs ruptured in this series. Rupture was described in three cases of PRCC (8.3%): it was spontaneous in two cases and resulted from a traffic accident in the third. CONCLUSIONS: The extensive necrosis regularly found in PRCC can cause rupture of the tumour followed by retroperitoneal bleeding. Rupture affected <10% of our cases of PRCC. CT findings are usually not characteristic and can mimic a simple haematoma of unknown origin. Similarly, the perioperative finding is unclear in most cases. The final correct diagnosis of the renal tumour is frequently established only by the pathologist.