CDC is a rare tumor cells derived from renal collecting ducts of Bellini, and has less than 1% renal malignancies. Reported by Mancilla-Jimenez et al. in its report on papillary tumors, the examination renal tissue distant from the tumor to appear, in some cases, atypical hyperplastic changes of collecting tubules.
This raised the possibility that some papillary tumors arose from distal tubular pithelium. Fleming and Lewi ago describe the detailed features of the CDC as pathological entity based on several case reports.
Pathology
This tumor is characterized by a core location, with a size ranging from 2 to 12 cm, the appearance of firm white-gray and irregular infiltrative edge. Grows radially from the renal hilum to invade the renal cortex, renal cap Sule, and the renal sinus. Histologically, an irregular pattern of growth embedded in a desmoplastic tubulopapillary stroma. The tubules are lined with cells with eosinophilic cytoplasm nail scarce. The cells display high-grade nuclei with brisk mitotic activity, and prominent nucleoli. Sometimes sarcomatoid changes or mucin can be seen. Molecular events and cytogenetic changes that contribute are not well characterized, and a different pattern has yet emerged. The immunohistochemical profile is variable, with generally positive for phytoagglutinins and high molecular weight cytokeratin, with coexpression of vimentin and negative for CD10 and villi.
Clinical Presentation
It is a very aggressive tumor, usually presenting at an advanced stage, with gross hematuria, abdominal pain / back pain and a flank mass. At diagnosis, they often have metastatic disease in the lung, liver, lymph nodes, bone, or adrenal gland. It is more common in men (ratio of about 2: 1) with a wide range of age groups (13-83 years with an
average 55 years). Computed tomography (CT), this tumor appears as a central mass arising infiltrating the preservation of the renal contour and minimal contrast enhancement. Patients may have generalized inflammatory symptoms secondary to the release of cytokines of the tumor and the inflammatory reaction associated with the tumor.
Treatment and Prognosis
The diagnosis of CDC is usually done after the operation, and unlike other radiological RCC is difficult and there is a low preoperative suspicion in view of the rarity of the disease. The prognosis is generally very poor with most patients with distant metastases developing rapidly, with a median survival of 22 months.
The role of nephrectomy has been the subject of debate due to frequent metastasis to the presentation. Radical nephrectomy in the context of metastasis CDC appears to be useful only for palliation. Based on the pathological, immunohistochemical and cytogenetic
similarity with urothelial (CTP), compared with conventional carcinomas clear cell RCC, the preferred approach in treatment of metastatic disease with chemotherapy has been in place for immunotherapy.
In the largest series reported Dimopoulos et al. subsequently reported the MD Anderson Cancer Center experience involving 12 patients with CDC treated from 1980-90. Seven of eight patients with metastatic disease were treated with different combinations of chemotherapy with doxorubicin and cisplatin, methotrexate; vinblastine (MVAC) regimen is the most common. Only one patient achieved a minor response lasting 5 months. Six patients were treated with a combination of interleukin-2 and IFN-α with a response in a patient. Peyromaure et al. reported two complete responses with cisplatin and gemcitabine combination chemotherapy, which lasted 9 and 27 months.
Radiotherapy in this series appeared to have minimal benefit for local recurrence. Chao et al. a review noted that some patients with regional nodal disease without distant etastases have long-term disease-free survival with adjuvant therapy.
While the overall benefit of chemotherapy or immunotherapy appears to be minimal, there seems to be a select group of patients who will benefit of these approaches. Cisplatin-Gemcitabine has significant activity with a favorable toxicity profile in urothelial cancers and has caused some significant response in patients with CDC. Thus access to the system to some extent, since the preferred first-line chemotherapy simply because it is less toxic than MVAC regimen, and no other major series of chemotherapy for collecting duct tumors has apparently better results.
This raised the possibility that some papillary tumors arose from distal tubular pithelium. Fleming and Lewi ago describe the detailed features of the CDC as pathological entity based on several case reports.
Pathology
This tumor is characterized by a core location, with a size ranging from 2 to 12 cm, the appearance of firm white-gray and irregular infiltrative edge. Grows radially from the renal hilum to invade the renal cortex, renal cap Sule, and the renal sinus. Histologically, an irregular pattern of growth embedded in a desmoplastic tubulopapillary stroma. The tubules are lined with cells with eosinophilic cytoplasm nail scarce. The cells display high-grade nuclei with brisk mitotic activity, and prominent nucleoli. Sometimes sarcomatoid changes or mucin can be seen. Molecular events and cytogenetic changes that contribute are not well characterized, and a different pattern has yet emerged. The immunohistochemical profile is variable, with generally positive for phytoagglutinins and high molecular weight cytokeratin, with coexpression of vimentin and negative for CD10 and villi.
Clinical Presentation
It is a very aggressive tumor, usually presenting at an advanced stage, with gross hematuria, abdominal pain / back pain and a flank mass. At diagnosis, they often have metastatic disease in the lung, liver, lymph nodes, bone, or adrenal gland. It is more common in men (ratio of about 2: 1) with a wide range of age groups (13-83 years with an
average 55 years). Computed tomography (CT), this tumor appears as a central mass arising infiltrating the preservation of the renal contour and minimal contrast enhancement. Patients may have generalized inflammatory symptoms secondary to the release of cytokines of the tumor and the inflammatory reaction associated with the tumor.
Treatment and Prognosis
The diagnosis of CDC is usually done after the operation, and unlike other radiological RCC is difficult and there is a low preoperative suspicion in view of the rarity of the disease. The prognosis is generally very poor with most patients with distant metastases developing rapidly, with a median survival of 22 months.
The role of nephrectomy has been the subject of debate due to frequent metastasis to the presentation. Radical nephrectomy in the context of metastasis CDC appears to be useful only for palliation. Based on the pathological, immunohistochemical and cytogenetic
similarity with urothelial (CTP), compared with conventional carcinomas clear cell RCC, the preferred approach in treatment of metastatic disease with chemotherapy has been in place for immunotherapy.
In the largest series reported Dimopoulos et al. subsequently reported the MD Anderson Cancer Center experience involving 12 patients with CDC treated from 1980-90. Seven of eight patients with metastatic disease were treated with different combinations of chemotherapy with doxorubicin and cisplatin, methotrexate; vinblastine (MVAC) regimen is the most common. Only one patient achieved a minor response lasting 5 months. Six patients were treated with a combination of interleukin-2 and IFN-α with a response in a patient. Peyromaure et al. reported two complete responses with cisplatin and gemcitabine combination chemotherapy, which lasted 9 and 27 months.
Radiotherapy in this series appeared to have minimal benefit for local recurrence. Chao et al. a review noted that some patients with regional nodal disease without distant etastases have long-term disease-free survival with adjuvant therapy.
While the overall benefit of chemotherapy or immunotherapy appears to be minimal, there seems to be a select group of patients who will benefit of these approaches. Cisplatin-Gemcitabine has significant activity with a favorable toxicity profile in urothelial cancers and has caused some significant response in patients with CDC. Thus access to the system to some extent, since the preferred first-line chemotherapy simply because it is less toxic than MVAC regimen, and no other major series of chemotherapy for collecting duct tumors has apparently better results.