Secondary renal amyloidosis due to primary Sjogren's syndrome: a case report.

Amyloidosis is a rare disorder characterized by the deposition of abnormal proteins in extracellular tissues, resulting in the dysfunction of vital organs and, eventually, death. The occurrence of amyloidosis due to primary Sjogren's syndrome (pSS) is a rare finding. This study describes a rare case of pSS complicated by amyloid-associated amyloidosis. Case presentation: A 35-year-old male was diagnosed with nephrotic syndrome and secondary amyloidosis caused by pSS. He had microscopic hematuria, a creatinine level of 6.59 mg/dl, and an elevated erythrocyte sedimentation rate of 107 mm/hrs. Furthermore, investigations of antinuclear antibodies, antimitochondrial antibodies, SSA, SSA native, and Ro-52 recombinant as well as rheumatoid factor showed positive results. After establishing the diagnosis of pSS through clinical, physical, and laboratory assessments, a renal biopsy was performed, which revealed the occurrence of secondary amyloidosis. Clinical discussion: The risk of developing secondary amyloidosis depends on the extent of elevated serum amyloid levels as well as persistent subclinical inflammation. The definitive diagnosis of amyloidosis requires histological confirmation of amyloid fibril deposition in tissue. Conclusion: Secondary renal amyloidosis is an unusual condition in patients with pSS. Still, it should be regarded in the differential diagnosis of patients with proteinuria and/or renal failure, and a renal biopsy should be performed.

Primary pancreatic synovial sarcoma: Report of a rare case and review of the literature.

Synovial sarcoma is a mesenchymal spindle cell tumor. Primary pancreatic sarcomas are extremely rare. The present study describes a rare case of synovial sarcoma in the head of the pancreas. A 35-year-old male presented with left upper quadrant abdominal pain. An endoscopic ultrasound examination revealed a complex solid-cystic lesion in the pancreatic head. He had undergone pancreaticoduodenectomy (Whipple procedure). A histological examination yielded negative results for AE1/AE3, CD10, S100, CD34, desmin, smooth muscle actin, ß-catenin, CD117, HMB45, chromogranin and synaptophysin. However, the results were positive for TLEI and vimentin, which is consistent with synovial sarcoma. Synovial sarcoma is a soft tissue malignant tumor. Primary pancreatic sarcomas frequently present as large, high-grade tumors in the pancreatic head. Histologically, there are several types of synovial sarcoma, such as monophasic, biphasic and poorly differentiated. A histological examination is necessary for the diagnosis as the imaging findings are not specifically suggestive of synovial sarcoma. The preferred course of treatment is complete resection with wide margins, followed by adjuvant chemotherapy and/or radiotherapy. Primary mesenchymal tumors of the pancreas are extremely uncommon. As a result, a diagnosis requires careful evaluation. Surgical resection is the main modality of treatment.

Synchronous ipsilateral papillary renal cell carcinoma and urothelial carcinoma: A case report.

Concurrence of renal cell carcinoma (RCC) and urothelial carcinoma (UC) in the same kidney is a rare phenomenon. It is critical to define this unusual disease to avoid a delay in diagnosis and improve the prognosis. The present study describes a case of a 71-year-old patient with synchronous ipsilateral RCC and UC of the renal pelvis and ureter. The patient presented with intermittent attacks of left loin pain with frank hematuria for 3 months and a weight loss of 5 kg over the same period of time. The patient had been a chronic heavy smoker for >45 years. Physical examination revealed stable vital signs; however, a mobile, non-tender mass was palpated in the left upper abdomen. A left nephroureterectomy with the removal of a bladder cuff was performed. Histopathological examination revealed a papillary RCC with a pathological stage of pT1N0Mx and a high-grade UC of the renal pelvis and ureter with a pathological stage of pT3-pN1-pMx. The postoperative recovery was good, and the patient was referred to an oncology center for further management. Previous reports have failed to identify definitive risk factors for the concurrence of RCC and UC. However, 24% of the patients in the various case reports in the literature were smokers. The most common presenting complaints included weight loss and painless hematuria. The concurrence of RCC and UC in the same kidney is a rare entity, and it frequently leads to a worse prognosis than the occurrence of RCC alone. Radical nephroureterectomy is the main line of treatment for patients with upper tract UC.

Pleomorphic giant cell carcinoma of the prostate: A case report and mini­review of the literature.

Pleomorphic giant cell carcinoma (PGCC) is an exceptionally uncommon form of prostate adenocarcinoma. It consists of unusually large and irregular cells with varied nuclei. The present study describes a rare case of prostatic PGCC. A 65-year-old male patient presented to the urology clinic with severe dysuria, nocturia, and frequent, urgent, and difficult urination for a period of 3 months. Pelvic magnetic resonance imaging revealed a large pelvic mass. A prostate biopsy was performed, and immunohistochemical analysis revealed positivity for the pan-epithelial markers, AE1/AE3, alpha-methyl acyl-CoA racemase, and focally for sphingolipid activator protein-2. While waiting for his pathology report, the patient's condition deteriorated, and he was diagnosed with intestinal obstruction. The patient underwent laparotomy and end colostomy. Later, he developed severe sepsis and wound dehiscence. After 2 weeks, the patient succumbed due to multiorgan failure. Prostatic PGCC cases are frequently associated with previous chemo-, hormone, or radiation therapy. Prior to the diagnosis of PGCC, it is critical to rule out urothelial carcinoma. Early recognition of this rare condition can lead to more effective therapy. Prostatic PGCC is extremely rare. Immunohistochemistry for prostatic markers, such as prostate-specific membrane antigen, prostate-specific antigen, NK3 homeobox 1 and androgen receptor, can be used to confirm its origin.

A huge retroperitoneal ganglioneuroma in a middle-aged patient: Report of a diagnostically challenging case with review of the literature.

Ganglioneuromas (GNs) are benign, slow-growing tumors of neural crest cell origin. On rare occasions, adipose cells have been detected in these tumors. The present study reported a case of a huge retroperitoneal GN misdiagnosed and mismanaged as a liposarcoma. A 54-year-old male patient presented with gradually progressing dull back pain with abdominal discomfort for the past 6 months. The patient had abdominal distention and mild right abdominal tenderness. Ultrasound of the abdomen and pelvis revealed a large right-sided retroperitoneal mass. Contrast-enhanced computed tomography findings were consistent with sarcoma. Cytologic examination was suggestive of liposarcoma. A decision was made to start neoadjuvant radiochemotherapy, which proved ineffective. Complete surgical excision of the mass was performed via a midline laparotomy. Histopathology of the mass confirmed the diagnosis of GN. At two years post-operation, the patient developed a recurrence in the subhepatic area with the same diagnosis and the recurrent mass was surgically removed. Initially, the imaging findings were not sufficiently specific to establish the diagnosis. Rarely, cytologic techniques have detected adipose cells in these tumors, resulting in misdiagnosis. Hence, histopathology is the gold standard for definitive diagnosis. Preoperative diagnosis of GN is difficult due to the lack of specific clinical manifestations, radiological confusion with other tumors and the presence of adipocytes in rare cases, leading to misdiagnosis and mismanagement.