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88 Cards in this Set
- Front
- Back
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What is the association between primary mediastinal germ cell tumors and hematologic malignancy?
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A syndrome of the two types of malignancy has been found only in patients with nonseminomatous mediastinal germ-cell tumors, particularly those with serologic or histologic evidence of yolk-sac elements. The two most common hematologic neoplasms seen in this syndrome are acute megakaryoblastic leukemia and malignant histiocytosis. Consistent cytogenetic abnormalities have not yet been identified, but the finding of the marker chromosome isochromosome(12p) in the mediastinal germ-cell tumor and associated leukemic blasts in one patient suggests that these tumors may arise from a common progenitor cel
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A patient with prostate cancer has significant enlargement of his recurrent primary tumor. What can account for a normal PSA in this setting?
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Prostate cancers can de-differentiate into small cell neuroendocrine tumors.
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Name 4 unusual locations where extragonadal germ cell tumors may arise.
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1. pineal gland
2. paranasal sinuses 3. liver 4. sacrum |
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Which sex chromosome disorder is associated with an increased risk for mediastinal germ cell tumors?
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Klinefelter syndrome (46/47, XXY, or XXY syndrome)
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In men with CIS of the testis, what is the incidence of invasive cancer at 5 years?
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50%.
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What chromosomal location is involved in 80% or more of germ cell tumors, and what other chromosomal abnormality is often present, in general terms, in these tumors?
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12p.
Hyperdiploidy is present in most germ cell tumors. |
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A 28 year old main presents with pain and swelling in his left testicle. How should he be managed initially?
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Antibiotics. If the symptoms persist at two weeks, an ultrasound is indicated.
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In general terms, how are seminomas different from non-seminomatous germ cell tumors?
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Seminomas retain totipotentiality and are exquisitely sensitive to chemotherapy and radiation.
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What are the four types of non-seminomatous germ cell tumors?
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1. choriocarcinoma
2. yolk sac tumor 3. teratoma 4. embryonal carcinoma |
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Which of the four types of nonseminomatous germ cell tumors can differentiate into the three other types?
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embryonal.
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A 30 year old man is diagnosed with testicular cancer. The pathology shows mostly seminoma with a small focus of embryonal carcinoma. How should he be treated?
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As nonseminoma.
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In questionable cases, how can seminoma and embryonal carcinomas be differentiated immunohistochemically?
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Seminomas are positive for placental AP and negative for LMW keratins; embryonal carcinoma almost universally expresses LMW keratins and are only positive for placental AP in 50% of cases.
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How does the lymphatic drainage differ for the right vs left testis? Cross metastasis usually goes which direction?
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Right sided tumors spread to the interaortocaval LNs, left sided tumors spread to the para-aortic LNs below the left renal artery and vein. Cross metastasis usually goes right to left.
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What are the half-lives of AFP and HCG?
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AFP = 5-7 days
HCG = 30 hours |
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Post-operative tumor markers, or markers followed during chemotherapy, which decline slowly are of what significance?
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They imply residual active disease and even in the absence of radiographic abnormalities have to be managed accordingly.
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Which street drug has been associated with elevation in HCG?
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Marijuana.
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How do serum tumor markers help determine the type of germ cell tumor?
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AFP = nonseminoma
HCG = both types |
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Elevations in any tumor marker are seen in what percentage of germ cell tumor by stage?
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Stage I = 205
Stage II = 40% Stage III = 60% |
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What are the high-risk factors in germ cell tumors?
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-degree of elevation of serum markers including LDH
-presence of nonpulmonary visceral metastases -medistinal primary |
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How is stage I seminoma treated?
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Radical inguinal orchiectomy + radiation to RP (para-aortic) nodes, +/- radiation to the ipsilateral pelvic LNs.
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What portion of patients with stage I seminoma, treated with the standard approach, will relapse and what is the usual time frame?
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20% will relapse usually after 15 months, but as far as 8-10 years out.
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How are stage IIA and stage IIB seminomas treated?
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radiation to RP and pelvic nodes with boost to gross disease. Prophylactic mediastinal radiation is not recommended.
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What percentage of patients with seminoma who relapse after radiation can be cured with chemotherapy?
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>90%
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A 23 year old man presents with painless swelling in his left testicle. A left radical inguinal orchiectomy and RPLND is performed. Pathology shows pure seminoma. Both AFP and HCG are moderately elevated. How should he be treated?
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He should be treated for nonseminomatous germ cell tumor.
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How are advanced germ cell tumors treated?
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Good risk gets 3 cycles of BEP, intermediate and high risk patients get 4 cycles.
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How are post-chemotherapy residual masses dealt with in the treatment of advanced germ cell tumors?
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Surgery (which is more difficult in seminomas) can be performed for lesions > 3 cm, or the patient can be followed with serial imaging studies.
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How stage I nonseminoma managed?
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After radical orchiectomy, surgery and RPLND are options. Risk factors that would prompt RPLND include vascular/lymphatic invasion, scrotal involvement or invasion of the tunica albuginea or spermatic cord, presence of embryonal carcinoma.
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What is the benefit of the conservative/surveillance approach to patients with stage I nonseminoma?
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Near 100% cure rate while sparing the 75% of patients with micrometastatic disease from unnecessary therapy.
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What is the likelihood of micrometastatic disease in patients with nonseminoma with any of the following: nodes > 2 cm in diameter, > 5 nodes involved, any extranodal extension.
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at least 50%.
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A 25 year old man has been diagnosed with stage II nonseminoma. He undergoes modified bilateral RPLND. Pathologically he is staged at I. How should he be managed? What if he was stage II pathologically?
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If he is compliant, the stage one patient can be followed closely. If he is stage II, and tumor markers fall after surgery by their expected half-lives, 2 cycles of chemo is appropriate. If the markers don't fall, then 3-4 cycles of chemo followed by resection of residual disease is indicated.
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A 37 year old man is diagnosed with a right testicle nonseminoma. Clinically he is staged IIB based on CT which shows two 3 cm lymph nodes. How should he be treated?
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Patients with stage IIB LNs (2-5 cm) or stage IIC (LNs >5 cm) are treated with primary chemotherapy (then surgery for residual). This is also true of patients with disease that appears to be unresectable regardless of size.
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In general, how is stage III nonseminoma managed?
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With cisplatin-based chemotherapy.
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Can carboplatin be substituted for cisplatin in the treatment of germ cell tumors?
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No. Efficacy would be compromised.
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What is the standard chemotherapeutic approach for good risk patients with germ cell tumor?
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THREE cycles of BEP OR
FOUR cycles of etoposide plus cisplatin. Etoposide must be given at 500 mg/m2 to maintain efficacy. |
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What is the standard chemotherapeutic regimen for intermediate-risk or high risk germ cell tumor?
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FOUR cycles of BEP
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What regimen is used as salvage in germ cell tumor?
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VIP
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What is the significance of late relapse (> 2 years) in patients with germ cell tumor?
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Late relapse is associated with resistance to salvage chemotherapy and overall poor prognosis.
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What is the role of the TIP (paclitaxel, ifofsamide, cisplatin) regimen in relapsed germ cell tumor?
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May be better for late relapses (compared to VIP) and in patients not appropriate for primary surgery.
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High-dose chemo with HSC support is used in patients with germ cell tumors who have an incomplete response to initial therapy or who relapse after salvage therapy. Which group of patients should not receive this treatment even if they otherwise fit criteria?
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Patients with primary mediastinal germ cell tumors.
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A 29 year old man undergoes orchietomy, RPLND and 3 cycles of BEP for yolk sac tumor of the left testis. Imaging at this time reveals residual disease at two sites, both > 2 cm in diameter. How should he be managed?
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Surgery to remove the residual tissue. If there is vital tumor and it can be completely resected, 2 more cycles of chemotherapy are indicated.
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Why should residual teratoma be resected after chemotherapy?
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Teratomas may grow and cause problems or transform to malignant tumors such as rhabdomyosarcoma or adenocarcinoma.
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Should patients with nonseminoma who present with initially visible disease but then have an apparent complete response to chemotherapy undergo surgery?
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This is controversial.
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What is the limitation of PET in determining the presence of residual disease after chemotherapy in patients with nonseminoma?
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PET can't tell teratoma from fibrosis.
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What is the one exception to the rule that tumor markers should return to normal after chemotherapy before surgery is undertaken?
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In patients who do not respond to salvage chemotherapy and have persistently elevated tumor markers, surgical resection of a solitary residual mass can be undertaken (by a specialist in this setting).
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What is the significance of finding i(12p) or excess 12p genetic material in tumors that have transformed from teratoma to somatic malignancy?
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Predicts response to cisplatin.
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Mediastinal nonseminomas are associated with what other types of malignancy?
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Hematologic cancers including myeloproliferative disorders, acute megakaryoblastic leukemia and malignant histiocytosis.
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Which organ system has been found to show signs of significant adverse effects related to the treatment regimens used in germ cell tumors?
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Cardiovascular, with increased risk of MI.
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What organism is associated with bladder cancer, where is prevalence highest, and what is histologic subtype of cancer?
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Schistosoma hematobium, Africa, squamous
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How well does staging bladder cancer by cystoscopy and biopsy compare with results after cystectomy?
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Only 50-60% concurrence.
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How is superficial bladder cancer (confined to mucosa or submucosa) managed?
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Transurethral resection followed by cystoscopy and urine cytology. Better surveillance techniques are being developed using, for example, FISH
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A patient with a history of superficial bladder cancer has a positive urine cytology 4 months after TUR of the initial lesion, but no abnormalities are seen on cystoscopy. What next?
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Selective catheterization and visualization of the upper urinary tracts.
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What is the role for Bacille-Calmette-Geurin (BCG) in treatment of bladder cancer?
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Indicated for the limitation of recurrence and to reduce the incidence of progression (for residual tumor or CIS). It is usually given in 6 weekly instillations, +/- maintenance. Surveillance for the upper urinary tract is necessary as well, as these surfaces get less exposure to the treatment.
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For bladder cancer lesions that do not response to BCG, what other agents are used (4)?
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Gemcitabine
Mitomycin C Doxorubicin Valrubicin |
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How is invasive bladder cancer treated?
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Radical systectomy with pelvic lymph node dissection (plus removal of urethra, prostate, uterus, ovaries...). Laparascopic and robotic techniques may make partial cystectomy feasible.
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About what percentage of patients are still alive at 5-10 years after radical cystectomy for muscle-invasive bladder cancer?
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68% and 66%
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What chemotherapy is used in metastatic bladder cancer?
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MVAC (MTX, vincristine, doxorubicin and cisplatin) or GC (gemcitabine, cisplatin). Efficacy is equal, MVAC more toxic in terms of immunosuppression. GC causes more thrombocytopenia and anemia.
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What is the role of adjuvant chemotherapy in the treatment of bladder cancer?
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There is no consensus, but it is reasonable to give MVAC or GC to patients with perivesicular invasion or lymph node involvement.
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What is the role of neoadjuvant chemotherapy in the treatment of bladder cancer?
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Neoadjuvant chemotherapy (usually GC) has shown a survival advantage and does represent the standard of care of invasive bladder cancer, but is grossly underutilized.
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What are the pros and cons to a bladder sparing approach to invasive bladder cancer?
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OS and DFS rates using trimodality therapy approach the rates achieved with cystectomy in some series, but the downside includes the need for ongoing surveillance (a third will recur, albeit most likely superficially) and radiation toxicity.
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What portion of patients are diagnosed with RCC based on incidental radiographic findings?
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25-50%.
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What portion of patients who undergo resection for localized RCC will recur?
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A third.
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Is tumor extension into the renal vein or IVC in RCC a contraindication to resection.
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No. Up to 50% of patients may have prolonged survival if the procedure is successful.
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What interventions, aside from nephrectomy, are available for small RCC lesions?
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nephron-sparing partial nephrectomy, cryo- or radio-frequency ablation.
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Does lymph node dissection prolong survival in RCC?
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No.
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What is the term that describes a patient with RCC, no detectable metastatic diseae, and evidence of hepatic dysfunction?
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Stauffer syndrome. The hepatopathy is often reversible with removal of the primary tumor.
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How should patients with RCC, in whom there is a high burden of symptoms or bleeding, be managed?
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Tumor debulking/nephrectomy.
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What type of RCC is associated with sickle trait?
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Medullary carcinoma.
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What is the genetic aberration in Von Hippel-Lindau disease?
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3p-. This condition is associated with clear-cell RCC, retinal angiomas, spinal cord hemangioblastomas and pheochromocytomas.
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What is the significance of sarcomatoid differentiation in RCC?
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It can occur in any of the 5 subtypes of RCC and is associated with a poor prognosis.
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Which of the 5 subtypes of RCC are the least aggressive?
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Oncocytomas are benign. Chromophobe tumors are typically indolent but can rarely metastasize (necrosis predicts a more aggressive phenotype).
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What is the prognosis for papillary renal cell cancers?
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Better than for clear cell RCC.
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How do collecting duct carcinomas behave compared to the other subtypes of RCC?
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These are very aggressive tumors. Patients with metastatic disease (50% at presentation) live for months only.
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What percentage of men over the age of 80 have prostate on autopsy?
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70%
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How does the Gleason score for grading prostate cancer work?
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Grading one a scale of 1 (most differentiated) to 5 (least differentiated), the scores of the two most prominent histologic specimens.
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What stage is prostate cancer with only PSA elevation?
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T1c
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A patient being followed for outflow symptoms and his physician follows the PSA. Initially the level is 2.8 and 6 months later is 3.7. What should be done next?
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Refer to a urologist; PSA velocity is >0.35 ng/mL/year. If the initial value is between 4 and 10, then a velocity of 0.75 ng/mL/year is used.
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How is the free PSA assay used?
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In patients with total PSA between 4-10 ng/mL, a free PSA less then 10% should prompt a biopsy, and less than 25% should prompt consideration of a biopsy.
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What strategy has been shown to reduce risk of prostate cancer?
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In randomized trials, 5-alpha-reductase (5-AR) inhibitors have been shown to significantly decrease the incidence of prostate cancer. However, no trials have demonstrated an impact of prostate cancer mortality, and the possibility that these agents increase the incidence of high-grade lesions cannot be excluded. (See '5-Alpha reductase inhibitors' above.)
The potential side effects (gynecomastia, decreased libido, erectile dysfunction), benefits (decreased symptoms from benign prostatic hyperplasia), and areas of uncertainty (impact on survival, long-term side effects) should be explained to men considering chemopreventive therapy |
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What findings should prompt MRI or CT in patients with newly diagnosed prostate cancer?
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PSA >20 ng/mL or high grade tumor (Gleason 7 or greater).
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What is the role of neoadjuvant hormonal therapy in prostate cancer?
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Neoadjuvant androgen deprivation prior to (and concurrent with) radiation has been of proven benefit. The same is not true of neoadjuvant ADT and surgery.
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For which patients with prostate cancer is a watch and wait approach appropriate?
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PSA < 10
Gleason 6 or lower T1c to T2a |
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What values in PSA kinetics suggest that a man would not benefit from salvage radiation after radical prostatectomy for prostate cancer?
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PSA velocity > 2 ng/mL/year prior to surgery.
Less than 3 year interval to PSA failure. Post-treatment PSA doubling time of less than 3 months. |
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Name 3 contraindications (relative) to GnRH agonists in prostate cancer patients.
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1. severe pain
2. urinary symptoms 3. spinal cord compromise |
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What is the definition of medically castrate?
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Serum testosterone <50 ng/mL.
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How is castrate-resistant prostate cancer that advances while on an anti-androgen treated?
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Initally by withdrawing the anti-androgen. A second hormone therapy, such as ketoconazole plus hydrocortisone, can be added, however this usually only results in 2-4 month responses.
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What is the standard first line chemotherapy for metastatic castration-resistant prostate cancer?
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Docetaxel 75mg/m2 Q 3 weeks
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What drug is commonly used as the second line chemotherapy agent in prostate cancer?
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Mitoxantrone
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What two-drug combination is approved for the treatment of painful bone metastases in prostate cancer?
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Mitoxantrone + Prednisone
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