Prognostic value of the neutrophil/lymphocyte ratio in enteropancreatic neuroendocrine tumors.

Accessible prognostic tools are needed to individualize treatment of neuroendocrine tumors (NETs). Data suggest neutrophil/lymphocyte ratios (NLRs) have prognostic value in some solid tumors, including NETs. In the randomized double-blind CLARINET study (NCT00353496; EudraCT 2005-004904-35), the somatostatin analog lanreotide autogel/depot increased progression-free survival (PFS) compared with placebo in patients with inoperable or metastatic intestinal and pancreatic NETs (grades 1-2, Ki-67 < 10%). The exploratory post-hoc analyses presented here evaluated the prognostic value of NLR in the CLARINET study cohort, in the context of and independently from treatment. Kaplan-Meier PFS plots were generated for patients with available NLR data, in subgroups based on NLR values, and 24-month survival rates were calculated. P values and hazard ratios for prognostic effects were generated using Cox models. 31216222 Baseline characteristics were balanced between lanreotide autogel/depot 120 mg (n = 100) and placebo (n = 101) arms. Irrespective of treatment, raw 24-month PFS rates were comparable across subgroups based on NLR tertiles [37.3% (low), 38.8% (middle), 38.8% (high); n = 67 per group] and NLR cutoff of 4 [38.1% (NLR ≤ 4; n = 176), 40.0% (NLR > 4; n = 25)]. Furthermore, NLRs were not prognostic in Cox models, irrespective of subgroups used. The therapeutic effect of lanreotide autogel/depot 120 mg was independent of NLRs (P > 0.1). These exploratory post-hoc analyses in patients with advanced intestinal and pancreatic NETs contrast with previous data suggesting NLR has prognostic potential in NETs. This may reflect the inclusion of patients with lower-grade tumors or use of higher NLR cutoff values in the current analysis.

Derived neutrophil to lymphocyte ratio as a prognostic factor in patients with advanced colorectal cancer according to RAS and BRAF status: a post-hoc analysis of the MRC COIN study.

The phase III Continuous or Intermittent (COIN) trial failed to show a benefit in overall survival (OS) of cetuximab in combination with chemotherapy for patients with metastatic colorectal cancer. High derived neutrophil to lymphocyte ratio (dNLR) has been shown to be prognostic in patients with metastatic colorectal cancer. The aim of this analysis is to evaluate dNLR as a predictive biomarker of the survival according to RAS and BRAF mutations status within the COIN trial. A post-hoc exploratory analysis of the COIN trial arms A and B was carried out. All patients with available white blood cell and neutrophil data were analysed. The dNLR was calculated using a formula that has previously shown predictive power in cancer patients: dNLR=ANC/(WBC-ANC). A high dNLR was defined as a value of 2.2 or more. dNLR was correlated with clinical outcomes using Kaplan-Meier and Cox regression analysis. A total of 1603 patients were assigned to the oxaliplatin-based chemotherapy (arm A, N=815) or oxaliplatin-based chemotherapy plus cetuximab (arm B, N=815) arms. There was a strong association between dNLR level and overall survival (OS) using Kaplan-Meier analysis. In all mutation groups, dNLR less than 2.2 was associated with better OS compared to dNLR of 2.2 or more. The median OS in patients with wild-type disease (dNLR<2.2 vs. dNLR≥2.2) was 22.8 versus 13.1 months [hazard ratio (HR)=1.33]; 16.9 versus 11.8 months (HR=1.36) in patients with RAS mutant tumours; and 12.6 versus 6.8 months (HR=1.67) in patients with BRAF mutant tumours. In patients with dNLR less than 2.2, the median OS was 19.2 months in arm A compared to 18.0 months in arm B (HR=1.11). Among patients with dNLR greater than or equal to 2.2, the median OS was 13.0 months in arm A compared with 13.1 months in arm B (HR=0.96). dNLR is strongly prognostic for survival in all mutation groups. dNLR does not predict for benefit from the addition of cetuximab.

Dexrazoxane added to doxorubicin-based adjuvant chemotherapy of breast cancer: a retrospective cohort study with a comparative analysis of toxicity and survival.

Dexrazoxane is indicated as a cardioprotective agent for patients receiving doxorubicin who are at increased risk for cardiotoxicity. Concerns have been raised on the use of dexrazoxane, particularly in adjuvant therapy, because of the risk of interference with the antitumor effect of doxorubicin. Two meta-analyses in metastatic breast cancer have rejected this hypothesis, but have shown an apparent increase in the severity of myelosuppression when dexrazoxane is used. Here, we analyzed retrospectively a cohort of our institute database to assess whether the addition of dexrazoxane causes more bone marrow suppression in breast cancer patients receiving doxorubicin-based adjuvant therapy. The secondary objectives were assessment of the incidence of febrile neutropenia, dose-schedule modifications, recorded cardiac events or cardiac test abnormalities, and overall survival. Eight hundred and twenty-two female patients who received adjuvant (or neoadjuvant) doxorubicin and cyclophosphamide for breast cancer between 2001 and 2013 were included. One hundred and four of these patients also received dexrazoxane concurrently with the adjuvant treatment. Hospital records and, when accessible, community clinic records were reviewed. The median follow-up duration was 7 years for patients receiving dexrazoxane and 7.5 years for patients not receiving dexrazoxane. 85.6% of patients were alive at data lock. Compared with the nondexrazoxane group, patients who received dexrazoxane were older (median age at diagnosis 59 vs. 52 years) and more likely to receive dose-dense AC therapy (73 vs. 59%) and adjuvant trastuzumab treatment (29 vs. 15%). Compared with the nondexrazoxane group, dexrazoxane treatment was associated with a higher rate of hematological side effects: leukopenia (48 vs. 39%), neutropenia (45 vs. 31%, P=0.003), anemia (86 vs. 73%, P=0.005), and thrombocytopenia (37 vs. 22%, P=0.001). There were more febrile neutropenia hospitalizations (20 vs. 10%, P=0.001) and dose reductions (22 vs. 8%, P<0.001) in the dexrazoxane group, but no significant difference in the incidence of treatment delays or cancellations. The incidence of cardiac events was the same in both treatment groups with and without dexrazoxane. There was a nonsignificantly lower mortality rate in the dexrazoxane group (9.6%) compared with the nondexrazoxane group (15.0%) at data lock. Adding dexrazoxane to doxorubicin in adjuvant therapy patients leads to higher rates of bone marrow suppression in all blood components, as well as more febrile neutropenia events, and dose reductions. No differences in events defined as cardiac toxicities were detected. Dexrazoxane had no detrimental effect on survival, despite the higher hematological toxicity, the older median age, and the higher prevalence of HER2-positive disease in the dexrazoxane group.

Derived neutrophil lymphocyte ratio is predictive of survival from intermittent therapy in advanced colorectal cancer: a post hoc analysis of the MRC COIN study.

BACKGROUND: The phase III COntinuous or INtermittent (COIN) trial failed to show non-inferiority of intermittent compared with continuous chemotherapy for advanced colorectal cancer in overall survival (OS). The present analysis evaluated whether the derived neutrophil to lymphocyte ratio (dNLR) could predict the effect of intermittent vs continuous chemotherapy on OS in patients with advanced colorectal cancer. METHODS: A post hoc exploratory analysis of COIN arms A and C was performed. Landmark analysis was conducted on all patients with available WBC and neutrophils data. The dNLR was calculated using a formula which has previously demonstrated predictive power in cancer patients: dNLR = ANC/(WBC-ANC). A high dNLR was defined using a cut-off value of ⩾ 2.22. Derived neutrophil to lymphocyte ratio was then correlated with clinical outcomes. Survival curves were generated based on dNLR using the Kaplan-Meier method. Comparison between groups was performed using Cox regression. RESULTS: A total of 1630 patients were assigned to the continuous (N = 815) or intermittent (N = 815) arms. There was a strong association between dNLR level and OS. The median survival times in the ITT population were 18.6 months and 12.5 months for patients with low and high dNLR, respectively (HR = 1.70; 95% CI = 1.52-1.90; P < 0.001). The estimate of the hazard ratio did not alter substantially (HR = 1.54) after adjusting for treatment, tumour status, number of metastatic sites, alkaline phosphate and platelet count. CONCLUSIONS: Derived neutrophil to lymphocyte ratio is strongly prognostic for survival in the COIN intermittent vs continuous treatment arms. Derived neutrophil to lymphocyte ratio does not predict for detrimental survival in patients treated with intermittent therapy.

Next-generation sequencing in patients with advanced cancer: are we ready for widespread clinical use? A single institute's experience.

The next-generation sequencing (NGS) assay targeting cancer-relevant genes has been adopted widely for use in patients with advanced cancer. The primary aim of this study was to assess the clinical utility of commercially available NGS. We retrospectively collected demographic and clinicopathologic data, recommended therapy, and clinical outcomes of 30 patients with a variety of advanced solid tumors referred to Foundation Medicine NGS. The initial pathologic examination was performed at the pathology department of the referring hospital. The comprehensive clinical NSG assay was performed on paraffin-embedded tumor samples using the Clinical Laboratory Improvement Amendments-certified FoundationOne platform. The median number of genomic alterations was 3 (0-19). The median number of therapies with potential benefit was 2 (0-8). In 12 cases, a comprehensive clinical NGS assay did not indicate any therapy with potential benefit according to the genomic profile. Ten of the 30 patients received treatments recommended by genomic profile results. In six of the 10 cases, disease progressed within 2 months and four patients died within 3 months of treatment initiation. Three of the 30 patients benefited from a comprehensive clinical NGS assay and the subsequent recommended therapy. The median PFS was 12 weeks (95% confidence interval 10-57) in patients treated with molecularly targeted agents chosen on the basis of tumor genomic profiling versus 48 weeks (95% confidence interval 8-38) in the control group treated with physician choice therapy (P=0.12). Our study suggests that NGS can detect additional treatment targets in individual patients, but prospective medical research and appropriate clinical guidelines for proper clinical use are vital.

The 21-gene recurrence score assay (Oncotype DX™) in estrogen receptor-positive male breast cancer: experience in an Israeli cohort.

OBJECTIVE: The 21-gene recurrence score (RS) assay has been widely adopted for use in early estrogen receptor (ER)-positive breast cancer to assess the risk for distant recurrence and the potential benefit of adjuvant chemotherapy. The primary aim of this study was to assess RS distribution in Israeli male breast cancer (MBC) patients. METHODS: The study population included 65 newly diagnosed Israeli MBC patients. Clinical and pathologic data were collected at the time of referral. Pathologic examinations were conducted at the pathology departments of the referring centers. The RS assay (Oncotype DX™) was performed on paraffin-embedded tumor samples at Genomic Health laboratories. RESULTS: The mean age of the patients was 65.1 years (range 38-88 years). Low-risk (RS<18), intermediate-risk (RS 18-30) and high-risk (RS≥31) scores were noted in 29 patients (44.6%), 27 patients (41.5%) and 9 patients (13.9%), respectively. The distribution of RS in male patients was similar to the distribution in 2,455 female patients from Israel referred during the same time period. CONCLUSION: Our data suggest that the distribution of Oncotype DX RS in ER-positive MBC patients is similar to that of female breast cancer patients.

Clinical manifestations and outcome of ANCA-related pauci-immune glomerulonephritis in patients with Sjögren's syndrome.

OBJECTIVES: Pauci-immune ANCA-related glomerulonephritis (GN) is extremely uncommon in patients with underlying Sjögren's syndrome (SS) and its clinical and laboratory characteristics have not been previously explored. METHODS: We carried out a thorough literature review in order to establish predisposing factors, clinical and laboratory manifestations, kidney biopsy features, treatment modalities and response to treatment of biopsy proven ANCA-related pauci-immune GN in patients with SS. RESULTS: From 1967 to 2011, seven patients with a mean age of 63±14.7 years were identified. The average duration of SS prior to development of pauci-immune GN was 50±62 months. A high incidence of extraglandular manifestations was identified: 50% had interstitial lung disease and/or peripheral neuropathy. All patients suffered from proteinuria (average 1397±905 mg per 24 hours) and haematuria at presentation. Almost 70% of patients suffered from severe anaemia (average haemoglobin 6.6±1.9 gr%). ANCA MPO was positive in all six patients, while ANCA PR3 was negative in all. All patients received corticosteroids and 70% received cyclophosphamide. One patient died and one patient developed end stage kidney disease. The rest of the study patients had improved renal function over time. CONCLUSIONS: Our study emphasises that patients with SS can present with atypical kidney pathology as ANCA-related GN. Thus, high clinical awareness is warranted to establish correct diagnoses. Given the powerful impact of kidney pathology on management of these patients and responsiveness to treatment demonstrated in our case series, the significance of timely diagnosis can not be overestimated.

[Intravenous vinorelbine as first line chemotherapy in patients with castration-resistant prostate cancer].

BACKGROUND: Docetaxel-based chemotherapy remains the treatment of choice in castration-resistant prostate cancer (CRPC). Generally, elderly patients poorly tolerate these drugs. Vinorelbine has a favolable toxicity profile and may be useful in elderly or unfit patients with castration-resistant prostate cancer. PURPOSE: The aim of this retrospective analysis was to evaluate the efficacy and safety of vinorelbine in patients with CRPC. PATIENTS AND METHODS: We analyzed the medical records of patients with CRPC treated in our institution with intravenous vinorelbine as first line chemotherapy. RESULTS: A total of 25 patients were assessable for efficacy. The median age was 73 years (range, 51-87 years); 9 out of 25 patients (36%) had a > 50% reduction in PSA levels from baseline. Mean progression-free survival was 7.2 months. Mean overall survival was 20.7 months. Mean overall survival for 11 patients treated with second-line taxane-based chemotherapy was 27.5 months compared to 16.8 months for patients who did not receive second-line chemotherapy. Treatment was generally well tolerated. CONCLUSION: On the basis of this small retrospective survey, we conclude that intravenous vinorelbine seems to be a therapeutic option in elderly or unfit patients with CRPC.

Efficacy of the CMFVP regimen in patients with metastatic breast cancer previously treated with anthracycline and taxane.

BACKGROUND: The aim of this retrospective study was to assess the efficacy and safety of the relatively old lowcost CMFVP regimen (cyclophosphamide, methotrexate, 5-fluorouracil, vincristine, prednisolone) as compared to newer and more costly chemotherapeutic agents. PATIENTS AND METHODS: An analysis of the medical records of female patients with metastatic breast cancers treated with a CMFVP combination at the Shaare Zedek Medical Center (SZMC) was performed. 32 patients with measurable metastatic disease were included in the analysis. The median age was 59.9 years (range 36-102 years). 20 of the patients had previously been treated with both anthracyclines and taxanes. RESULTS: Therapy was generally well tolerated and toxicity manageable. Median time to disease progression was 20.97 weeks (range 2-103 weeks). 6 patients (19%) demonstrated a partial response, and 7 patients (22%) had stable disease lasting 3 or more months for an overall clinical benefit of 41%. 4 of the 20 patients previously treated with both anthracyclines and taxanes had a partial response, and 4 patients had stable disease lasting 3 or more months for a clinical benefit of 40%. CONCLUSION: On the basis of this small retrospective survey, we conclude that CMFVP combination chemotherapy is both effective and safe in patients with metastatic breast cancer previously treated with both taxanes and anthracyclines.

Tumor lysis syndrome in a patient with merkel cell carcinoma and provoked pathologic sequence of acute kidney injury, reduced clearance of carboplatin and fatal pancytopenia.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare, highly malignant cancer of the skin primarily affecting the elderly, with a tendency for local recurrence and regional lymph node metastasis. It is very unusual for this kind of tumor to induce clinically apparent tumor lysis syndrome (TLS) which is a consequence of spontaneous cytolysis or massive tumor cell lysis, beginning a few hours after the initiation of treatment. CASE REPORT: We report here on a patient with metastatic MCC, who developed TLS following combination chemotherapy with carboplatin and etoposide. CONCLUSION: The evolving acute kidney injury (AKI) provoked a pathologic sequence of reduced renal clearance leading to protracted clearance of carboplatin and subsequent fatal pancytopenia. When AKI occurs in close association with the administration of carboplatin, the institution of rescue hemodialysis is recommended to decrease plasma carboplatin levels and avoid this lethal complication.

Indwelling central venous access port insertion during bevacizumab-based therapy.

Indwelling central venous catheters and implantable port systems are widely used in the care of patients with cancer. Bevacizumab, a humanized monoclonal antibody against vascular endothelial growth factor, significantly prolongs survival when added to intravenous 5-fluorouracil-based chemotherapy as first-line treatment for metastatic colorectal cancer. It has also been shown to be of value in a range of other malignant diseases. Some elements of the toxicity profile of bevacizumab, however, such as bleeding and impaired wound healing, could interfere with surgical procedures involved in the treatment of the diseases. The aim of this study was to evaluate the possible effect of bevacizumab in increasing the morbidity associated with an indwelling central venous access port in patients currently receiving the drug, or those who had received it in the preoperative run-up to surgery. An analysis of the medical records of 57 patients with a variety of cancers, who had received an indwelling central venous access port, either during the course of treatment with bevacizumab or in the 4-week period before the commencement of therapy was carried out, with particular emphasis on periprocedural complications. Eight of the patients also had diabetes mellitus. There were no instances of delay in wound healing, abnormal bleeding, or wound infection in any of the patients and no episodes of skin ulceration during bevacizumab treatment. Although this is a relatively small study, and no definitive conclusions can be drawn at this stage, our data suggest that an indwelling central venous access port insertion may be carried out shortly before or during bevacizumab treatment without increasing periprocedural morbidity.

Monitoring long-term treatment with pegylated liposomal doxorubicin: how important is intensive cardiac follow-up?

Pegylated liposomal doxorubicin (PLD; Doxil or Caelyx) has been shown to be as effective as conventional doxorubicin, and to have a significantly better cardiac safety profile. The aim of this study was to assess the safety of delivering doses exceeding 700 mg/m of PLD to patients with solid tumors. A review of the medical records of 149 patients with a variety of solid tumors treated with PLD was performed. The findings in 12 patients who had reached or exceeded cumulative doses of 700 mg/m (median=1.071 mg/m, range 712-1856 mg/m) were reviewed. Changes in left ventricular ejection fraction (LVEF), and in clinical cardiac status were analyzed. The median age of the patients was 53.9 years and the median follow-up from the start of PLD treatment was 44.6 months. None of the 12 patients had clinical congestive heart failure secondary to cardiomyopathy. Seven of the 12 patients underwent further assessment of LVEF by echocardiography or multiple gated acquisition scan, which revealed a stable or improved ejection fraction.PLD is cardiac safe for long-term treatment of metastatic solid tumors. Its maximal cumulative dose remains undefined. Frequent determinations of LVEF, as routinely done for other anthracyclines, do not appear to have any clinical value in patient follow-up. In metastatic patients with no evidence of cardiac risk factors, it may be sufficient to measure LVEF at baseline.

Long-term survival in a patient with non-small cell lung cancer presenting as small intestinal obstruction.

BACKGROUND: Despite the early and widespread dissemination of nonsmall cell lung cancer, clinically significant metastases in the small bowel are rare, and when they do occur, the patient is almost always in the terminal stages of the disease. CASE REPORT: We report on a patient who presented initially with small bowel obstruction resulting from metastatic spread of a squamous cell carcinoma of the lung. Following surgical removal of the affected bowel, the patient underwent radiofrequency ablation of the lung tumor and subsequent lower lobe resection. The patient continues to be free of disease 2 years from the initial diagnosis. CONCLUSIONS: This report highlights the need for a timely diagnosis of an acute surgical abdomen and for the inclusion of small intestinal metastases in the differential diagnosis of the acute abdomen in a patient with a lung cancer. Resection of the obstructing tumor and postoperative systemic therapy should be considered. In cases of isolated metastases, resection of the primary lung tumor is indicated.

Reinduction of hormone sensitivity to goserelin following chemotherapy with vinorelbine in castration-resistant prostate cancer.

Primary androgen ablation leads to symptomatic improvement and a reduction in prostate-specific antigen (PSA) serum levels in patients with advanced prostate cancer, but all patients eventually become refractory to hormone therapy with progression of the disease and a life expectancy of about a year. We describe a patient who developed castration resistance, was treated with vinorelbine, and continues to be progression free on therapy with luteinizing hormone releasing hormone agonists alone, more than 2.5 years following cessation of treatment with vinorelbine.

Combination therapy with oxaliplatin and 5-fluorouracil in a patient with severe hepatic dysfunction associated with metastatic adenocarcinoma of the large bowel.

Oxaliplatin has been shown to be valuable in the treatment of patients with colorectal cancer. Many of these patients will develop liver metastases during the course of their disease, with, in some cases, severe hepatic dysfunction. Although single agent oxaliplatin can be administered safely in patients with severely compromised liver function (as it is not metabolized by the liver), little is known of its safety in these patients when administered in the preferred combination with 5-fluorouracil (which is metabolized by the liver) and leucovorin (FOLFOX protocol). We report on a very sick patient with major liver dysfunction, a bilirubin of 11.2 mg/dl (190 micromol/dl) and an open abdominal wound, for whom palliative hospice care alone was originally proposed, who responded dramatically to the combination. His bilirubin fell to 0.6 mg/dl (10.2 micromol/dl) and his liver function tests returned to near normal levels. The combination was well tolerated and clinical improvement continued for more than 11 months before disease progression was observed.

Long-term response to pegylated liposomal doxorubicin in patients with metastatic soft tissue sarcomas.

Doxorubicin and ifosfamide are currently considered the cornerstones of treatment for advanced soft tissue sarcomas (STSs). Pegylated liposomal doxorubicin (PLD) has been shown to have equivalent activity to doxorubicin and an improved toxicity profile. A review of the medical records of 11 patients with a variety of STSs treated with PLD was performed. The median age of the patients was 54.8 years. Of the 11 patients, seven received no earlier systemic therapy for their sarcoma. The initial dose per course was 40-60 mg/m2 every 4 weeks with dose reduction to 40 mg/m2 in the second or third cycle. A median of 11 cycles was given (range, two to 29 cycles). Treatment was generally well tolerated. We did observe some toxic effects as described earlier with PLD, including mild myelosuppression, skin toxicity and fatigue. No cardiotoxicity was observed. Of the 11 treated patients, six had a partial response, two had a best response of stable disease and three had progressive disease. All six patients with a partial response had an extended time to progression. To date, two patients continue on treatment (15 and seven cycles); one patient has stable disease 60 months after withdrawal of PLD (after eight cycles) and one patient had progression of disease 7 months after the withdrawal of therapy after 20 cycles. Of the two patients with stabilization of their disease, one had progression after 29 months and one continues on treatment for 6 months. PLD is active and safe for long-term treatment of metastatic STSs and may be important in maintaining response.

Mitomycin C and vinblastine: an active regimen in previously treated breast cancer patients.

BACKGROUND: Metastatic breast cancer has a substantial mortality burden on women worldwide. Presented herein is our experience with the combination of mitomycin-C and vinblastine in heavily pretreated breast cancer patients. METHODS: Candidates were women with measurable metastatic disease, previously exposed to two or more chemotherapy regimens. Mitomycin-C was given at the dose of 10 mg/m2 on day 1 and vinblastine at 6 mg/m2 on days 1 and 21 of each 42-day cycle. Analysis included patients exposed to one or more cycles of therapy. Kaplan-Meier curves were used to generate overall survival and time-to-treatment progression curves. RESULTS: Forty patients previously exposed to a median of three prior regimens were included. Partial response and stable disease were reported in 14 (35%) and 10 (25%), patients, respectively, for a clinical benefit of 60%. With a median follow-up of 11 months, the median time to progression and survival durations lasted 4 and 12 months, respectively. In a subgroup of 17 women with prior anthracycline and taxane exposure, partial response and stable disease were reported in 4 (23.5%) and 5 (29%), respectively. Treatment was generally well tolerated, with grade 3-4 hematologic and non-hematologic toxicity reported in 8 (20%) and 3 (7.5%) patients, respectively. Two cases of fatalities (5%) occurred with pulmonary toxicity in women heavily exposed to mitomycin-C (cumulative doses of > or = 40 mg/m2) and soon after red blood cell transfusion. CONCLUSIONS: Chemotherapy with mitomycin-C and vinblastine is active and well-tolerated in heavily pretreated breast cancer patients. Caution should be taken to avoid blood transfusion alone with mitomycin-C therapy.

Staphylococcus aureus bacteremia secondary to severe erlotinib skin toxicity.

Epidermal growth factor receptor (EGFR) inhibitors are associated with unique and dramatic dermatologic side effects. Skin rash is the dose-limiting factor for all EGFR inhibitors and is usually dose related and reversible. Microbiologic stains and cultures from skin rash usually do not show an infectious cause. We report on a patient with known non-small-cell lung cancer who developed Staphylococcus aureus bacteremia secondary to severe erlotinib skin toxicity.

Primary amelanotic melanoma of the vagina.

BACKGROUND: Primary malignant melanoma of the vagina is extremely rare, accounting for 0.3-0.8% of all malignant melanomas. True amelanotic vaginal melanoma showing no melanin on histological examination is exceedingly rare, accounting for only 2% of all vaginal melanomas. CASE REPORT: We describe a 31-year-old female patient who presented with locally advanced amelanotic melanoma of the vagina, with no evidence of metastatic spread on the computerized tomography (CT) scan, but who was subsequently diagnosed as suffering from metastatic disease by positron emission tomography (PET)-CT performed a few weeks following posterior pelvic exenteration. CONCLUSION: Specific immunohistochemical staining with melanoma markers should be performed to confirm or exclude a diagnosis of amelanotic melanoma in all patients presenting with a vaginal mass composed of undifferentiated epithelioid malignant cells. Fluorodeoxyglucose (FDG)-PET-CT should be performed as part of the preoperative evaluation, to identify the presence or absence of metastatic disease in all patients with vaginal melanoma.