Epithelioid sarcoma is an uncommon mesenchymal malignancy which represents less than 1% of all sarcomas. Rarer still are reports of this tumor initially presenting in the vulva. We report a case of vulvar proximal-type epithelioid sarcoma.

A 52-year-old had a 5-month history of slowly growing papule on the right labia majora. Excision of the mass revealed a tumor composed of large polygonal cells with abundant eosinophilic cytoplasm. An immunohistochemistry panel revealed cytokeratin AE1/AE3 positivity only. She underwent radical vulvectomy with bilateral groin node dissection. The specimen revealed a cream tan, firm, fairly defined mass at the right vulva. Microscopic examination showed a sheet-like growth pattern of large pleomorphic epithelioid cells with large vesicular nuclei and prominent nucleoli. The tumor showed loss of INI1 nuclear expression and absence of CD34 staining. EMA was positive. The case was signed out as proximal-type epithelioid sarcoma of the right vulva. Two months post-operatively, the patient was given concurrent chemotherapy with 5 cycles of cisplatin 40 mg/m2 and 6600 centigray vulvar intensity-modulated radiotherapy. She had no evidence of disease for five months until repeat workup showed tumor recurrence in the perineum. She was subsequently given 6 cycles of gemcitabine 900 mg/m2 and gemcitabine 900 mg/m2 with docetaxel 100 mg/m2. Two months after, repeat workup showed persistent progressive disease in the vulva. She was subsequently given 4 cycles of doxorubicin 60 mg/m2 and is for repeat workup.

The immunohistomorphologic features of this tumor, in addition to its unusual location, present a diagnostic challenge. Clues to the diagnosis include an initial presentation as a soft tissue mass and microscopic features showing the presence of epithelioid to spindle cytomorphology with an infiltrative growth pattern. Immunohistochemistry studies revealing the loss of INI1 nuclear expression and expression of epithelial markers would ultimately establish the diagnosis of this rare clinical entity.

Human ; Female ; Middle Aged: 45-64 Yrs Old ; Female Urogenital Diseases ; Vulvar Neoplasms ; Epithelioid Sarcoma

: Epithelioid sarcoma is an uncommon mesenchymal malignancy which represents less than 1% of all sarcomas. Rarer still are reports of this tumor initially presenting in the vulva. We report a case of vulvar proximal-type epithelioid sarcoma. : A 52-year-old had a 5-month history of slowly growing papule on the right labia majora. Excision of the mass revealed a tumor composed of large polygonal cells with abundant eosinophilic cytoplasm. An immunohistochemistry panel revealed cytokeratin AE1/AE3 positivity only. She underwent radical vulvectomy with bilateral groin node dissection. The specimen revealed a cream tan, firm, fairly defined mass at the right vulva. Microscopic examination showed a sheet-like growth pattern of large pleomorphic epithelioid cells with large vesicular nuclei and prominent nucleoli. The tumor showed loss of INI1 nuclear expression and absence of CD34 staining. EMA was positive. The case was signed out as proximal-type epithelioid sarcoma of the right vulva. Two months post-operatively, the patient was given concurrent chemotherapy with 5 cycles of cisplatin 40 mg/m2 and 6600 centigray vulvar intensity-modulated radiotherapy. She had no evidence of disease for five months until repeat workup showed tumor recurrence in the perineum. She was subsequently given 6 cycles of gemcitabine 900 mg/m2 and gemcitabine 900 mg/m2 with docetaxel 100 mg/m2. Two months after, repeat workup showed persistent progressive disease in the vulva. She was subsequently given 4 cycles of doxorubicin 60 mg/m2 and is for repeat workup. The immunohistomorphologic features of this tumor, in addition to its unusual location, present a diagnostic challenge. Clues to the diagnosis include an initial presentation as a soft tissue mass and microscopic features showing the presence of epithelioid to spindle cytomorphology with an infiltrative growth pattern. Immunohistochemistry studies revealing the loss of INI1 nuclear expression and expression of epithelial markers would ultimately establish the diagnosis of this rare clinical entity.
epithelioid sarcoma ; vulvar neoplasms ; female urogenital diseases

Every year in Korea about 50,000 terminally ill patients pass away in pain and more than 90% of the elderly oppose medical treatment to keep terminally ill patients alive. In medical ethics, the patient's right to self-determination is important, but he or she is unconscious or older and cannot express himself/herself, then his/her family should decide whether or not prolong treatment. From February 4, 2018, Hospice-palliative care and self-determination life sustaining decision-making Act will extend the life of the terminally ill patient. A specialist in the treatment of genitourinary cancer, focuses on prevention and treatment of cancer. However, by understanding this law, including terminology and how to implement of process of decision and stop life-sustaining treatment, a uro-oncologist has to counsel and perform education for terminally ill patients. A revision law has been forwarded to simplify the procedure. The Assembly should make haste with reviewing the law to help reduce the pain of terminally ill patients and their families.
Aged ; Comprehension ; Decision Making ; Education ; Ethics, Medical ; Hospice Care ; Humans ; Jurisprudence ; Korea ; Palliative Care ; Patient Rights ; Specialization ; Terminally Ill ; Urogenital Neoplasms

PURPOSE: Incidence of cancer has been increasing each years. The incidence of genitourinary cancer are also being increased. We aim to describe the time trends in genitourinary cancer incidence in Jeju province. MATERIALS AND METHODS: Age-standardized rates for incidence of genitourinary tract cancer in Korea and world standard population were calculated using the databases from the Jeju Cancer Rigistry from 1999 to 2012. RESULTS: Cancer incidence in Jeju was increased approximately 2-fold from 1217 cases 1999 to 2376 cases in 1999. Prostate cancer occurred most frequently followed by bladder tumor, and kidney cancer in 2012. In the prostate cancer, incidence was increasing every year from 18 cases in 1999 to 110 cases in 2012. Age-standardized incidence rate (ASR) was approximately four time increased, from 3.0 cases in 1999 and 11.2 cases in 2012. Incidence of bladder cancer were 43 cases in 1999 and 27 cases in 2012, ASR of bladder cancer was increased from 5.0 cases in 1999 to 7.5 cases in 2012. Kidney cancer showed constantly reducing the incidence. Peak is 60 cases in 2009 and recent incidence was 37 cases in 2012. There was no significant change in the incidence of testicular cancer, ureter cancer, renal pelvis cancer from 1999 to 2012. CONCLUSIONS: Incidence of cancer has been increasing in Jeju, similar to other area In Korea. Prostate cancer is the most common urological cancer in Jeju province, and kidney cancer incidence is recently decreasing. We should try to prevent genitourinary tract cancer caused obesity and smoking.
Incidence* ; Kidney Neoplasms ; Kidney Pelvis ; Korea ; Obesity ; Prostatic Neoplasms ; Smoke ; Smoking ; Testicular Neoplasms ; Ureteral Neoplasms ; Urinary Bladder Neoplasms ; Urogenital Neoplasms* ; Urologic Neoplasms

The incidence of cancer is sharply increasing. Cancer is a leading cause of death as well as a significant burden on society. The incidence of urological cancer has shown a higher than average increase and will become an important concern in the future. Therefore, an overall and accurate understanding of the incidence of urological cancer is essential. In this study, which was based on the Korea National Cancer Incidence Database, annual incident cases, age-standardized incidence rates, annual percentage change (APC), and distribution by age group were examined in kidney, bladder, and prostate cancers, respectively. From 1999 to 2011, the total number of each type of urological cancer was as follows: kidney cancer (32,600 cases, 25.5%), bladder cancer (37,950 cases, 29.7%), and prostate cancer (57,332 cases, 44.8%). The age-standardized incidence rates of prostate cancer showed a significant increase with an APC of 12.3% in males. Kidney cancer gradually increased with an APC of 6.0% for both sexes and became the second most frequent urological cancer after 2008. Bladder cancer showed no significant change with an APC of -0.2% for both sexes and has decreased slightly since 2007. The distribution of kidney cancer according to age showed two peaks in the 50- to 54-year-old and 65- to 69-year-old age groups. Bladder and prostate cancers occurred mostly in the 70- to 74-year-old age group. The proportions of male to female were 2.5:1 in kidney cancer and 5.6:1 in bladder cancer. We have summarized the incidence trends of kidney, bladder, and prostate cancers and have provided useful information for screening and management of these cancers in the future.
Female ; Humans ; Incidence ; Kidney Neoplasms/epidemiology ; Male ; Prostatic Neoplasms/epidemiology ; Republic of Korea/epidemiology ; Urinary Bladder Neoplasms/epidemiology ; Urogenital Neoplasms/*epidemiology

Primary seminal vesicle (SV) adenocarcinoma is a rare tumor. A small amount of data about the role of imaging to detect tumor recurrence is available. We report the case of a 58-year-old patient with primary SV clear-cell well-differentiated adenocarcinoma. Clinical and instrumental examinations were negative for the 32 months after treatments when computed tomography scan, [18F]fluoro-D-glucose positron emission tomography/computed tomography and pelvic magnetic resonance imaging showed the appearance of a lesion in the left perineal muscle suspected for recurrence. Patient was symptomless. Cytology of the suspected lesion confirmed SV adenocarcinoma recurrence. The combined approach, using radiological and nuclear medicine techniques, seems to be effective in the follow-up of SV adenocarcinoma. Technological advances, together with awareness of this rare tumor, have the potential of improving patients outcomes not only by providing earlier detection and accurate staging, but also by detecting recurrence and thereby avoiding delays and therapeutic dilemmas.
Adenocarcinoma* ; Diagnosis* ; Electrons ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Multidetector Computed Tomography ; Nuclear Medicine ; Positron-Emission Tomography ; Recurrence* ; Seminal Vesicles* ; Urogenital Neoplasms

BACKGROUND: Performance of antibody screening and identification tests before blood transfusion is important because the unexpected presence of red cell antibodies may cause hemolytic transfusion reactions. Many patients with malignancy undergo transfusion in order to overcome pancytopenia due to disease itself or chemotherapy. We investigated the type distribution of unexpected red cell antibodies in cancer patients and compared our results with those of other institutions. METHODS: From January 2008 to June 2011, 30,989 serum samples were screened using a LISS/Coombs card and ID-DiaCell I, II (DiaMed AG, Morat, Switzerland). Data-Cyte Plus Reagent Red Blood Cells (Medion Diagnostics, Dudingen, Switzerland) were used in performance of antibody identification tests. RESULTS: Out of 30,989 serum samples, 180 cases (0.58%) showed screening-positive results, and unexpected antibodies were identified in 72 cases. The type of unexpected antibody observed most often in cancer patients was a member of the Rh antibody group, anti-E in 17 cases (29.8%), followed by anti-Lea in five cases (8.8%) and anti-e in three cases (5.3%). While Rh group antibodies were observed in the colon cancer group, non-Rh group antibodies were observed in the rectal cancer group. And, in the genitourinary cancer group, Lewis group antibodies were more frequently detected than others. CONCLUSION: Findings from our study demonstrated a type distribution of unexpected red cell antibodies that was similar to those reported in previous studies. Compared with non-cancerous patients, no difference in type distribution of unexpected red cell antibodies was observed in cancer patients. Some antibodies were frequently observed in certain cancer groups. Further comprehensive research on unexpected antibodies based on location or histologic type of cancer is needed.
Antibodies ; Blood Group Incompatibility ; Blood Transfusion ; Colonic Neoplasms ; Erythrocytes ; Humans ; Mass Screening ; Pancytopenia ; Rectal Neoplasms ; Urogenital Neoplasms

Granular cell tumors are uncommon soft tissue neoplasm of nerve sheath origin, which are predominately benign. Granular cells can be found at any site in the body including the tongue, skin, subcutaneous tissue, breast, gastrointestinal, and urogenital systems. However, granular cell tumors have only been rarely described in the chest wall. Here we report a case of a granular cell tumor that occurred in the chest wall of a 59-year-old woman, along with a review of the literature.
Breast ; Female ; Granular Cell Tumor ; Humans ; Middle Aged ; Skin ; Soft Tissue Neoplasms ; Subcutaneous Tissue ; Thoracic Wall ; Thorax ; Tongue ; Urogenital System

BACKGROUNDPrimary Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of urogenital tract is a rare condition with non-specific clinical presentations, which can make it difficult to diagnose. In this study, we summarize the clinical presentation, pathological features, therapeutic strategies, and prognosis of ES/PNET.

METHODSClinical information on two cases of ES/PNET in the penis and ureter was analyzed, and relevant literature was reviewed.

RESULTSES/PNET was confirmed pathologically, immunohistochemically and via molecular biology techniques in the penis (n=1) and ureter (n=1). In one case, a tumor was found at the base of penis, which had invaded the corpus cavernosum, and resulted in a massive enlargement of the penis. This tumor was initially diagnosed as an endocrine disorder. However, a confirmed diagnosis was made 11 months later when massive metastases in both lungs were noted. A tumor biopsy was performed to confirm the diagnosis, and chemotherapy with a CAV (cyclophosphamide+doxorubicin+vincristine)+IE (ifosfamide+etoposide) regimen for 9 months was prescribed. In the second case, a child was admitted due to abdominal pain and a hydroureter in the right kidney, as determined by ultrasonography. A tumor was found in the right ureter at the level of iliac vessels. Removal of the tumor and ureteral anastomosis were performed, and chemotherapy with CAV+IE for 8 months were prescribed. Both patients are currently being followed-up closely.

CONCLUSIONSES/PNET is a highly malignant tumor and has poor prognosis. Pre-operative diagnosis of ES/PNET of urogenital tract is difficult and largely depends on pathology, immunohistochemistry, and, if applicable, molecular biology. Comprehensive therapy may include surgery, chemotherapy and radiotherapy.

Child ; Child, Preschool ; Humans ; Male ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; surgery ; Sarcoma, Ewing ; diagnosis ; surgery ; Urogenital Neoplasms ; diagnosis ; surgery

PURPOSE: To evaluate the clinicopathological characteristics and prognosis of localized resectable genitourinary sarcomas in adults. MATERIALS AND METHODS: Between September, 1996 and November, 2008, 18 consecutive cases of adults (12 men and 6 women; median age 48.8 years) who were treated for primary genitourinary sarcomas were identified. The following variables were analyzed: patient age, gender, body mass index, American Society of Anesthesiologists (ASA) score, primary organ, tumor histology, size, necrosis, Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade, and surgical margin positivity. Recurrence-free survival and disease-specific survival were the study end-points. RESULTS: The most common presenting symptom was a palpable mass (six cases, 33.3%), the most common site was the kidney (six cases, 33.3%), and the most common histological subtype was leiomyosarcoma (eight patients, 44.4%). Complete resection with negative surgical margins was achieved in 13 patients (72.2%). The median follow-up period was 49.9 months (range 6.4 to 147.6). The recurrence-free survival rates at 1, 3, and 5 years were 81.6%, 66.5%, and 66.5%, respectively. Recurrence-free survival only associated significantly with ASA score (p=0.018). The disease-specific survival rate at 1, 3, and 5 years was 88.9%, 76.2%, and 67.7%, respectively. Disease-specific survival was associated significantly only with FNCLCC grade (p=0.042). CONCLUSION: Although genitourinary sarcomas in adults are a rare group of tumors with a poor prognosis, some patients may have a favorable prognosis. Our findings suggest that FNCLCC grade is the most important prognostic factor for these patients.
Adult ; Aged ; Female ; Humans ; Immunohistochemistry ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Neoplasm Grading ; Prognosis ; Republic of Korea ; Sarcoma/metabolism/pathology/*surgery ; Urogenital Neoplasms/metabolism/pathology/*surgery ; Young Adult