RESUMEN
INTRODUCTION: Benign cystic tumors represent only 2% of all pancreatic tumors (pancreatic cancer - PC). In contrast to malignant cystic tumors, these tumors occur typically in young women. A solid pseudopapillary tumor is a relatively rare affliction representing less than 4% of cystic PC. Although the tumor is considered benign, metastasis, especially to the spleen, has been reported in approximately 0.5-4% patients. Despite R0 resection, vascular and perineural invasion is monitored in 20% of cases. Invasion is the cause of tumor relapse in up to one third of affected patients. Characteristic features of the disease are latent clinical indicators such as signs of pain and malfunction of intestinal passage. The diagnostics is based on MR, sometimes in combination with positron emission tomography. Medical treatment is specifically surgical. CASE HISTORY: Authors present a case of a 20-year-old female patient who was examined due to pain in the epigastrium, further exasperated by a voluminous expansion of the abdominal cavity. An initial ultra-sonographic examination was conducted to examine for possible nodular focal nodular hyperplasia of the liver; however, an MRI scan revealed the likelihood of a malignant tumor in the subhepatic region. During laparotomy, a tumor protruding from the head of the pancreas was discovered and removed. Histological examination showed it was a solid pseudopapillary pancreatic tumor. After a month of good post-operative progress, the patient was re-operated because of the presence of pancreatic fistula. Complete healing of the fistula was achieved after total parenteral nutrition and administration of sandostatin. At her last examination, the patient was without any problems. CONCLUSION: Solid pseudopapillary pancreatic tumors are rare, mainly benign lesions. It is essential to consider them in the differential diagnostics of afflictions of the subhepatic region, especially in young women. The only generally accepted cure nowadays is surgical resection. It is necessary to monitor patients consistently considering the rather high frequency of relapse of disease despite R0 resections. In the case of surgical removal, the 5-year survival rate is near 97%. Key words: solid pseudopapillary tumor of pancreas - diagnostics - therapy The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 17. 4. 2018 Accepted: 13. 8. 2018.
Asunto(s)
Neoplasias Pancreáticas/diagnóstico , Enfermedades Raras/diagnóstico , Adulto , Femenino , Fármacos Gastrointestinales/uso terapéutico , Humanos , Imagen por Resonancia Magnética , Octreótido/uso terapéutico , Fístula Pancreática/diagnóstico , Fístula Pancreática/terapia , Neoplasias Pancreáticas/cirugía , Nutrición Parenteral , Enfermedades Raras/cirugía , Ultrasonografía , Adulto JovenRESUMEN
Thyroidization of kidney reminiscent of thyroid follicles with accumulation of inspissated colloid-like material in renal tubules is a hallmark of chronic pyelonephritis. We identified 6 tumors in the kidney, distinct from currently known subtypes of renal cell carcinoma, with a striking histology that closely mimicked well-differentiated thyroid follicular neoplasms and raised the possibility of metastatic follicular thyroid carcinoma. Three occurred in males and 3 in females with an age range of 29 to 83 years and size range from 1.9 to 4 cm. All tumors were encapsulated and exclusively demonstrated follicular architecture comprising of microfollicles and macrofollicles containing inspissated colloid-like material. A minor component of small tightly packed follicles devoid of secretions was also noted. The follicles were lined by cells with moderate amphophilic to eosinophilic cytoplasm with round nuclei and occasional prominent nucleoli. The tumors were nonimmunoreactive with thyroglobulin and thyroid transcription factor 1 and for markers contemporarily used for renal differentiation. The tumors had a gene expression profile distinct from clear cell and chromophobe renal cell carcinoma. Comparative genetic hybridization failed to reveal cytogenetic alterations. Mean follow-up of 47.3 months (range: 7 to 84 mo) showed that 5 patients had no evidence of disease and 1 developed a metastasis to the renal hilar lymph nodes in which the follicular architecture with colloid was retained. Thyroid-like follicular renal cell carcinoma represents a unique histologic subtype of renal cell carcinoma of low malignant potential and its primary importance is to distinguish it from metastatic carcinoma from the thyroid.