“…While renal carcinoid tumors show no distinctive pattern on computed tomographic or magnetic resonance imaging, and can present as well-circumscribed, non-enhancing or slightly enhancing masses, a large tumor in a predominantly medullary location with lack of central necrosis should raise suspicion for extrapulmonary small cell carcinoma of the kidney (12). Small cell carcinoma of the kidney has been reported to occur in conjunction with urothelial carcinoma (13), but none of the six patients in our review had any evidence of urothelial carcinoma.…”
Background
Renal neuroendocrine tumors (NET), comprising carcinoid tumors and small cell carcinomas, are a rare group of neoplasms. The rarity of these tumors poses a diagnostic and therapeutic challenge. Our purpose was to characterize the cases treated at a tertiary cancer center and to evaluate patients' outcomes with the available treatment modalities.
Methods
This is a retrospective study of patients with renal NET seen at The University of Texas MD Anderson Cancer Center between January 1, 2001, and January 1, 2011. Patient and tumor data were analyzed by descriptive statistical methods.
Results
Three cases of carcinoid tumors and six cases of small cell carcinoma were identified. The median age at diagnosis was 53 years for patients with carcinoid and 65 years for patients with small cell carcinoma. The most common presenting symptoms were back pain, flank pain, and hematuria. The morphological appearance of the tumor cells and their immunohistochemical reactivity for neuroendocrine markers and cytokeratin helped establish the diagnosis. Nephrectomy was the mainstay of treatment for carcinoid tumors, yielding good long-term results, even in the presence of metastases. Surgery and chemotherapy were utilized for small cell carcinoma of the kidney. The median overall survival for patients with small cell carcinoma of the kidney was 17.3 months.
Conclusion
Renal carcinoid tumors are indolent and are associated with prolonged survival, while small cell carcinomas of the kidney are aggressive tumors with relatively short overall survival. Although palliative in nature, cytotoxic chemotherapy is the mainstay of therapy and is best given before surgery.
“…While renal carcinoid tumors show no distinctive pattern on computed tomographic or magnetic resonance imaging, and can present as well-circumscribed, non-enhancing or slightly enhancing masses, a large tumor in a predominantly medullary location with lack of central necrosis should raise suspicion for extrapulmonary small cell carcinoma of the kidney (12). Small cell carcinoma of the kidney has been reported to occur in conjunction with urothelial carcinoma (13), but none of the six patients in our review had any evidence of urothelial carcinoma.…”
Background
Renal neuroendocrine tumors (NET), comprising carcinoid tumors and small cell carcinomas, are a rare group of neoplasms. The rarity of these tumors poses a diagnostic and therapeutic challenge. Our purpose was to characterize the cases treated at a tertiary cancer center and to evaluate patients' outcomes with the available treatment modalities.
Methods
This is a retrospective study of patients with renal NET seen at The University of Texas MD Anderson Cancer Center between January 1, 2001, and January 1, 2011. Patient and tumor data were analyzed by descriptive statistical methods.
Results
Three cases of carcinoid tumors and six cases of small cell carcinoma were identified. The median age at diagnosis was 53 years for patients with carcinoid and 65 years for patients with small cell carcinoma. The most common presenting symptoms were back pain, flank pain, and hematuria. The morphological appearance of the tumor cells and their immunohistochemical reactivity for neuroendocrine markers and cytokeratin helped establish the diagnosis. Nephrectomy was the mainstay of treatment for carcinoid tumors, yielding good long-term results, even in the presence of metastases. Surgery and chemotherapy were utilized for small cell carcinoma of the kidney. The median overall survival for patients with small cell carcinoma of the kidney was 17.3 months.
Conclusion
Renal carcinoid tumors are indolent and are associated with prolonged survival, while small cell carcinomas of the kidney are aggressive tumors with relatively short overall survival. Although palliative in nature, cytotoxic chemotherapy is the mainstay of therapy and is best given before surgery.
“…Since the first reported case of renal carcinoid tumour over 40 years ago, only 56 cases have been published in English‐language reports [4–6]. Primary renal SCC not associated with renal pelvic urothelial carcinoma is even rarer, with 16 cases reported in 12 studies [7–12]. No renal LCNEC has been reported to date.…”
“…A smoking history is present in some cases [2]. Imaging analysis may disclose a large heterogeneous renal mass frequently with extension into the renal vein or inferior vena cava and/ or metastasis to lymph nodes [7,11]. An association with papillary RCC and chromophobe RCC has been reported [2,12].…”
Section: Clinical Characteristicsmentioning
confidence: 99%
“…The tumor usually presents as a huge mass when discovered, and extends into the perinephric adipose tissue [3,[6][7][8][9][10][11]15]. The cut surface of the tumor shows white, gray-white or gray-yellow color [10,16,17].…”
Section: Macroscopic Findingsmentioning
confidence: 99%
“…Multilobulation is frequently seen [18]. Invasion into the inferior vena cava or renal vein and metastasis to lymph nodes are frequent [3,6,7,10,11,15]. Direct invasion into adjacent anatomical structures such as liver, diaphragm or abdominal wall may be observed [2,6,17].…”
Small cell carcinoma (SmCC) of the kidney is extremely rare. In this article, we present a review of SmCC of the kidney with the focus on clinical and pathobiological aspects. Macroscopically, this tumor often shows a bulky mass extensively replacing the renal parenchyma with vascular invasion and metastasis to lymph nodes. Histologically, the tumor is composed of small cells with scant cytoplasm, round to oval nuclei, finely granular chromatin and inconspicuous nucleoli. Rosette or tubular formation may be present. Immunohistochemically, neoplastic cells show variable positivity for neuron-specific enolase, chromogranin A, synaptophysin, CD57 (Leu7) and CD56. A dot-like staining pattern for cytokeratin may also be observed. An electron microscopic examination may identify electron-dense neurosecretory granules in the cytoplasm. As a therapeutic option, nephrectomy and systemic chemotherapy should be considered. However, despite multimodal therapy, most patients have a dismal outcome and die of widely metastatic disease within one to two years. As there are limited genetic data on SmCC of the kidney, a large series studying this will be needed in the future.
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