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Romuald Curík

Publications and source records attributed to Romuald Curík.

4 recordsLinked to original sources

Elejalde syndrome--a case report.

Elejalde syndrome (McKusick 200995), also known as acrocephalopolydactylous dysplasia, is a rare condition. We describe a sixth patient with this syndrome which is characterized by craniosynostosis and hyperproliferation of fibroblasts in many tissues including skin, liver, kidney, and pancreas. The cause of the syndrome is the homozygous state of an autosomal recessive mutation. We present a hypothesis that Elejalde syndrome might be associated with an inactivating FGFR gene mutation.

Abnormalities, Multiple↗

Detection of sentinel lymph nodes in cervical cancer. A comparison of two protocols.

BACKGROUND: The aim of this study was lymphatic mapping to identify SLN in cervical cancer (CaCerv) with radioactive colloids, intraoperative detection with patent blue dye (PBD) and gamma probe (GP) and biopsy and comparison of two protocols. MATERIAL AND METHODS: In 54 patients with CaCerv before hysterectomy and lymph nodes dissection (LND) we performed preoperative lymphoscintigraphy utilizing 99mTc-colloid (Nanocoll, SentiScint or Nanocis), activity 40 MBq, on the operation day (30 women) or the day before operation (24 women). Gynaecologists injected 4 peritumoural injections of colloid into the cervix around the tumour. Scintigraphy followed 25-50 minutes (one-day protocol) or 12-19 hours (two-day protocol) after injection. Gynaecologists also injected 4 peritumoural injections of PBD into the cervix around the tumour. All women underwent SLN biopsy and LND (in average 35 lymph nodes were taken) and hysterectomy. SLNs (active and/or blue lymph nodes) were examined by a pathologist [histopathology and immunohistochemistry (IH) with detection of cytokeratine]. No SLN was examined without IH. RESULTS: The gynaecologists withdrew 123 SLNs (on average 2.27/1 patient) and in total 1898 lymph nodes (on average 35/1 patient). In 1 woman the tumour was inoperable. Two-day protocol, which involved scintigraphy, PBD and GP detected SLNs on both sides (45 SLNs) in 17 women (70.8%), SLNs on the one side (6 SLNs) in 3 patients (12.5%) and no SLNs were found in 4 women (16.7%). One-day protocol detected SLNs on both sides in 23 patients (74.1%)--63 SLNs, in 7 women on one side (25.9%)--9 SLNs. Metastases in SLNs (with or without metastases in other LN) were found in 21 patients (38.9%)--in 1 woman of stage FIGO IB1, in 1 woman of stage FIGO IB2, in 1 patient of stage FIGO IIIA and in all 18 patients of stage FIGO IIIB. False negative SLN detection was 0%. CONCLUSIONS: In SLN detection in patients with CaCerv, all 3 methods--scintigraphy, PBD and GP--should be used, and the success rate of SLN detection increases, although scintigraphy has lower significance than in SLN detection in malignant melanoma and breast cancer. One-day protocol had a better detection rate of SLN than two-day protocol. The method is promising but its results are not as unequivocal and optimistic as in breast cancer and malignant melanoma, and it is still experimental. Additional experience is necessary.

Adult↗

Urothelial signet-ring cell carcinoma of the renal pelvis with collagenous spherulosis: a case report.

We present a unique case of urothelial carcinoma of the right renal pelvis. It occurred in a 58-year-old woman. The tumor was located in the renal pelvis with extension into the adjacent renal medulla and cortex. Two years after surgical excision the patient is free of recurrence and metastasis. The tumor was well demarcated, without capsule, firm, solid, and whitish on the cut surface. It was 3x4 cm in largest diameter and without signs of necroses and hemorrhages. The tumor did not infiltrate the ureter. Histologically the predominant pattern of the tumor was adenocarcinomatous differentiation, and only very rare foci of urothelial carcinoma composed of typical transitional cells were found. No signs of intestinal type of metaplasia and adenocarcinoma, changes similar to the cystitis cystica or cystitis glandularis, were found in the tumor or in its vicinity. Most of the tumor looked like solid nests composed of cells with intracytoplasmic lumens. The resulting appearance was that of typical signet-ring cell change. These solid nests were usually surrounded by columnar epithelium, which in many areas formed papillary structures. A very striking feature was formation of collagen spherules. Small collagen spherules were often surrounded by a layer of the neoplastic cells so that collagenous rosettes were formed. In some areas these collagenous spherules clustered together so that they formed areas of collagenous spherulosis. The collagen in the spherules reacted positively with collagen IV. Ultrastructurally these spherules were formed by basal membrane-like material. Intracytoplasmic lumens of the signet-ring cell change were endowed by slender microvilli at ultrastructural level.

Carcinoma, Signet Ring Cell↗

Renal pelvic carcinoma with unusual appearance simulating amyloidosis (myeloma kidney): a report of five cases.

We describe 5 cases of urothelial carcinoma (UC) of the renal pelvis, which grew in a distinctive gross and microscopical pattern into the renal parenchyma. Five patients (2 men and 3 women, mean age 67.4 years) underwent nephrectomy for vague clinical findings. The cut surface of the tumor was white to light gray and the consistency was elastic. The corticomedullary border was indistinct, resulting in an appearance that suggested amyloidosis or myeloma. The renal pelvis showed normal mucosa with areas of dysplastic changes. The tumors spread from the renal pelvis in a diffuse and irregular, infiltrative pattern and surrounded intact glomeruli. Detailed sampling of invasive tumor component showed foci of UC with transitions to clear squamous cells. The predominant clear squamous neoplastic cells had foci of granular eosinophilic cytoplasm and resembled conventional renal cell carcinoma. Four patients were alive and without signs of the disease for 5 months to 4 years after nephrectomy; 1 died of generalized tumor 7 months after nephrectomy. The unusual gross and microscopic features result in varied problems in differential diagnosis, which are discussed herein.

Aged↗