Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: a case report and literature review.

Mixed adenoneuroendocrine carcinoma (MANEC) is defined as a tumor composed of both adenocarcinoma and neuroendocrine components. Here, we report the case of a 75-year-old woman with ampullary MANEC. She visited a physician with the chief complaint of dark urine and was diagnosed with advanced jaundice. Subsequently, she was referred to our hospital. Contrast-enhanced computed tomography scan revealed a neoplastic lesion measuring approximately 2 cm with a contrast effect at the duodenal papilla. Upper endoscopy showed a non-exposed tumor at the duodenal papilla. After biliary drainage, a subtotal stomach-preserving pancreaticoduodenectomy was performed. Histopathological examination revealed that the tumor components were composed of circular-to-oval atypical cells admixed with tubular adenocarcinoma tissue. These atypical cells were immunohistochemically positive for synaptophysin and diagnosed as neuroendocrine carcinoma with a Ki-67 labeling index of 63%. The patient was diagnosed with MANEC with a neuroendocrine carcinoma component of approximately 40%. The neuroendocrine carcinoma component had metastasized to the posterior pancreatic lymph nodes. Despite starting adjuvant chemotherapy with S-1, computed tomography revealed the presence of multiple liver metastases within 4 months after surgery. MANEC with neuroendocrine carcinoma is well known to have an extremely poor prognosis. Therefore, establishing a multidisciplinary therapy including chemotherapy is crucial.

Carcinosarcoma (adenocarcinoma, neuroendocrine carcinoma, undifferentiated carcinoma and chondrosarcoma) of the gallbladder.

We present an extremely rare case of carcinosarcoma with 4 different tumor components in an 88-year-old female. After a diagnosis of acute cholecystitis, we performed percutaneous transhepatic gallbladder drainage in the patient without success, followed by a cholecystectomy and choledocholithotomy. The mass was a 60 × 25 mm polypoid lesion of the gallbladder identified histologically as a carcinosarcoma with adenocarcinoma, neuroendocrine carcinoma, undifferentiated carcinoma and chondrosarcoma components. The biliary-type adenocarcinoma portion exhibited acinar growth patterns with columnar cells having large and markedly hyperchromatic nuclei. These tumor cells were immunohistochemically positive for MUC1 and CDX2. The neuroendocrine carcinoma, small cell type, cells were densely packed and small, with scant cytoplasm, finely granular nuclear chromatin and absence of nucleoli. The mitotic index was high. These tumor cells were immunohistochemically positive for synaptophysin, Ki-67 (index 40%), MUC1, CDX2 and c-Kit. The undifferentiated carcinoma consisted exclusively of spindle cells containing large, markedly hyperchromatic nuclei with a high mitotic index. These tumor cells were immunohistochemically positive for AE1/AE3. The chondrosarcoma was composed of blue-gray chondroid matrix and atypical chondrocytes containing large, hyperchromatic nuclei. These tumor cells were immunohistochemically positive for S100. Its attributes might be suggestive of a greater malignant potential and pathogenesis of carcinosarcoma.

Adult-Onset Mixed Germ Cell Tumor Composed Mainly of Yolk Sac Tumor Around the Pineal Gland: A Case Report and Review of the Literature.

BACKGROUND: Mixed germ cell tumors (MGCTs) usually occur in children. In the present report, we discuss an extremely rare case of adult-onset MGCT composed mainly of yolk sac tumor (YST) around the pineal gland. CASE DESCRIPTION: A 54-year-old Japanese man presented with disturbance of consciousness, Parinaud's syndrome, and gait disturbance. Magnetic resonance imaging revealed a pineal mass lesion, and subtotal resection of the tumor was achieved. The histologic diagnosis was MGCT, consisting mainly of YST. Although he underwent 5 courses of chemotherapy and craniospinal irradiation after surgery, tumor dissemination could not be controlled, and he died 10 months postoperatively. CONCLUSION: The present case highlights the need for clinicians to include YST in the differential diagnosis of acute progressive lesions around the pineal region, even in adult patients.

Mixed adenoneuroendocrine carcinoma (MANEC) of the gastroesophageal junction: a case report and review of the literature

Esophageal cancer is the fourth most common neoplasm of the gastrointestinal tract. It is responsible for 1.7% of all deaths related with cancer. The two main types of esophageal cancer are squamous cell carcinoma and adenocarcinoma. Other types of esophageal cancer are uncommon. We present a 57-year-old man admitted to the hospital with nausea and vomiting due to a high-grade malignant mixed adenoneuroendocrine carcinoma of the gastroesophageal junction. The patient underwent Ivor-Lewis esophagectomy and adyuvant chemoradiotherapy. At 8-month follow-up he was alive without evidence of recurrence (AU)

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Laparoscopic Treatment of Mixed Malignant Ovarian Germ Cell Tumor in a 16-Year-Old Female Adolescent.

BACKGROUND: Malignant ovarian germ cell tumors are rare entities, although they account for a large proportion of ovarian masses in young women. These tumors have traditionally been removed via laparotomy, because of their large size and solid nature. The use of laparoscopy for treatment of adnexal masses in adolescents has been heavily debated and poorly studied to date. CASE: A 16-year-old female patient presented with abdominal pain and an 11-cm adnexal mass on ultrasound. An emergent laparoscopic salpingo-oophorectomy was performed without complication. Pathology revealed a mixed malignant ovarian germ cell tumor. SUMMARY AND CONCLUSION: Laparoscopic fertility-sparing surgery offers many benefits over laparotomy, and should be considered in cases of young women with large adnexal masses, even if potential for malignancy exists.

Overexpression of CIP2A promotes bladder cancer progression by regulating EMT

Background: Bladder cancer is the second most common urological malignancy worldwide. CIP2A is a newly identified inhibitor of PP2A. Recent studies have highlighted a potential role for CIP2A in promoting the proliferation of several cancer cells. However, the role of CIP2A in bladder cancer still remains unclear. Methods: The expression of CIP2A was detected by quantitative real-time polymerase chain reaction and IHC in bladder cancer tissues and bladder cancer cell lines. In addition, silencing of CIP2A with siRNA was performed in T24 cells, and the impact on proliferation, and apoptosis of T24 cells was analyzed. Results: Our results found that CIP2A expression levels were higher in bladder cancer tissues and cell lines. Furthermore, CIP2A siRNA significantly reduced the proliferation rate of T24 cells, induced a significant population of early and late apoptosis, and could reverse EMT in T24 cells, indicates that CIP2A expression is increased in bladder cancer and implies a role of the protein in bladder cancer progression. Conclusions: These results suggest that CIP2A is involved in tumor progression, and thus CIP2A could represent selective targets for the targeted treatments of bladder cáncer (AU)

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Lesión linfoproliferativa del saco lagrimal: caso clínico / Lacrimal sac lymphoproliferative lesion: Case report

CASO CLÍNICO: Se presenta el caso de una mujer de 51 años con tumoración firme a nivel del canto interno del ojo derecho de 5 años de evolución. La biopsia excisional mediante dacriocistectomía estableció el diagnóstico de lesión linfoproliferativa de bajo grado (hiperplasia linfoide reactiva). DISCUSIÓN: Los tumores del saco lagrimal son muy raros, con un pico de incidencia en la quinta década de la vida. La clínica en fases iniciales es parecida a la obstrucción lagrimal por otras causas, de ahí que hasta un 40% de estos tumores no se sospechen y sean diagnosticados durante la realización de una dacriocistorrinostomía

CASE REPORT: The case is presented of a 51 year-old woman with a firm mass at the medial canthus of the right eye of five years onset. A low-grade lymphoproliferative lesion (reactive lymphoid hyperplasia) was diagnosed from an excisional biopsy. DISCUSSION: Lacrimal sac tumors are rare, with a peak incidence in the fifth decade of life. The initial clinical features are epiphora and medial canthus swelling. As it mimics nasolacrimal duct obstruction, up to 40% of these tumors are misdiagnosed until undergoing surgery

Malignant mixed Mullerian tumor of broad ligament with synchronous ovarian and endometrial carcinoma: a rare association.

Extragenital malignant mixed Mullerian tumor (MMMT) is a rare tumor in females, and it is even more rarely encountered among the multiple genital malignancies. There are some reports of extragenital MMMTs associated with synchronous or metachronous gynecologic tumors of Mullerian duct origin. We recently encountered an MMMT of broad ligament which is associated with papillary serous cystadenocarcinoma of the ovary and endometrioid adenocarcinoma arising in atypical polypoid adenomyoma endometrium in a 76-year-old woman. This case is presented for its rarity and unique presentation. To our knowledge, ours is the first reported case of this unique combination of multiple synchronous genital malignancies.

Hipercalcemia humoral secundaria a carcinoma mixto de endometrio / Humoral hypercalcemia in mixed endometrial carcinoma

La hipercalcemia secundaria a enfermedad neoplásicaes una entidad frecuente causada en la mayor partede los casos por secreción ectópica de PTHrp. A pesarde esto hay ciertos tumores, como los carcinomas uterinos,en donde este tipo de manifestación paraneoplásicaestá muy poco descrita. Presentamos un caso dehipercalcemia humoral en un carcinoma mixto de endometrio (AU)

Hypercalcemia secondary to neoplastic diseaseis a frequent entity caused in the majority of cases byectopic secretions of PTHrP. Despite this there are certaintumours, such as uterine carcinomas, in which thistype of paraneoplastic manifestation has been describedvery little. We present a case of humoral hypercalcemiain a mixed endometrial carcinoma (AU)

Exophytic benign mixed epithelial stromal tumour of the kidney: case report of a rare tumour entity.

BACKGROUND: Mixed epithelial and stromal tumour (MEST) represents a recently described benign composite neoplasm of the kidney, which predominantly affects perimenopausal females. Most tumours are benign, although rare malignant cases have been observed. CASE REPORT: A 47-year-old postmenopausal female presented to the urologist with flank pain. A CT scan of the abdomen showed a 30-mm-in-diameter uniform mass adjacent to the pelvis of the left kidney. Surgical exploration showed a tumour arising from the lower anterior hilus of the left kidney. The tumour could be excised by preserving the kidney. By intraoperative frozen section the tumour showed characteristic features of MEST with epithelial-covered cysts embedded in an "ovarian-like" stroma. Additional immunohistochemistry investigations showed expression for hormone receptors by the stromal component of the tumour. DISCUSSION: MEST typically presents in perimenopausal women as a primarily cystic mass. Commonly, the tumour arises from the renal parenchyma or pelvis. The tumour is composed of an admixture of cystic and sometimes more solid areas. The stromal cells typically demonstrate an ovarian-type stroma showing expression for the estrogen and progesterone receptors. CONCLUSION: MEST represents a distinctive benign tumour entity of the kidney, which affects perimenopausal woman. The tumour should be distinguished from other cystic renal neoplasms. By imaging studies it is difficult to distinguish between a benign or malignant nature of the tumour. Thus, intraoperative frozen section is necessary for conservative surgery, since the overall prognosis is favourable and renal function can be preserved in most cases.

Clinical and bone density outcomes of tumor-induced osteomalacia after treatment.

OBJECTIVE: To report the outcomes of tumor-induced osteomalacia after treatment, particularly related to recovery of bone mass. METHODS: We review the clinical course of a 61-year-old man extremely debilitated from multiple fractures and neuromuscular weakness due to tumor-induced osteomalacia and report the changes in biochemical markers and bone density after removal of the causative neoplasm. RESULTS: At the time of diagnosis, the patient's serum phosphorus and 1,25 dihydroxyvitamin D levels were depressed, and his fibroblast growth factor-23 level was markedly elevated. These values normalized 2 days after surgery and remained within their respective reference ranges 4 and 12 months after resection of a mesenchymal tumor. Lumbar bone density values (T-scores) were 0.445 g/cm2 (-5.9) preoperatively, 0.939 g/cm2 (-1.4) 4 months after surgery, and 1.152 g/cm2 (0.7) 12 months after surgery. Left femoral neck values at the same time points were 0.525 g/cm2 (-3.0), 1.035 g/cm2 (-0.8), and 1.184 g/cm2 (1.9). Ultra-distal radius values at the same time points were 0.128 g/cm2 (-7.0), 0.191 g/cm2 (-5.9), and 0.259 g/cm2 (-4.8). In addition, he recovered neuromuscular function and was able to leave his wheelchair. CONCLUSION: Tumor-induced osteomalacia can be an extremely debilitating disease. With successful localization, identification, and resection of the neoplasm, bone mass and physical function can recover.

Subependymoma revisited: clinicopathological evaluation of 83 cases.

OBJECT: Subependymomas are rare ependymal neoplasms. To date, a large clinicopathologic study of these benign neoplasms treated with modern neurosurgical techniques has not been reported. METHODS: Eighty-three cases of subependymoma were retrieved from the files of the Armed Forces Institute of Pathology. Clinicopathological features were reviewed; chromogenic in situ hybridization analysis for chromosome 22 was performed (n = 8), and patient follow-up was obtained (n = 34). Overall, the patients included 68 males and 15 females, 1.5 to 85 years of age (mean, 51.0 years). Twenty-seven cases were discovered at autopsy and the remaining were surgical specimens (n = 56). Tumors arose in the posterior fossa (n = 43), lateral ventricles (n = 37), spinal cord (2) and only one arose in the temporal horn. Tumors ranged in size from 2.0 mm to 60 mm in greatest dimension (mean, 23.0 mm). Eighteen-percent (15/83) of subependymomas exhibited a mixed histologic pattern; that is, subependymoma together with another glial tumor. The most common mixture (13/15) was subependymoma and ependymoma. Surgical excision was used in all symptomatic patients; 10 patients received radiation. Four patients developed a recurrence due to incomplete excision. All patients were without evidence of disease at the last follow-up: alive (n = 28) or dead (n = 8). CONCLUSIONS: Age is the only variable found to be significantly associated with survival. Currently, surgical methods result in an excellent long-term clinical outcome. Subependymomas do not appear to be associated with NF2 mutations.

Puberdade precoce associada a tumor misto ovariano (células germinativas- estroma-cordão sexual): aspectos clínicos, diagnósticos e manejo de um caso / Granulosa-stromal tumor of the ovary: a case of mixed germ cell-cord stromal tumor of fhe ovary with endocrinological considerations

Os tumores de estroma ovariano são responsáveis por 5 a 8 por cento dos tumores ovarianos. Sua manifestação clínica inicial mais comum em meninas pré-puberais é o desenvolvimento de puberdade precoce. Neste artigo discutimos o caso de uma menina de 7 anos e 2 meses com tumor de estroma ovariano do tipo misto - cordões-sexuais-células germinativas -, cuja manifestação inicial foi puberdade precoce isossexual, de evolução rápida e progressiva. Os níveis séricos de testosterona, estradiol e 17alfaOH-progesterona encontravam-se elevados. A ecografia pélvica-abdominal demonstrou massa anexal direita. Salpingo-ooforectomia unilateral foi realizada com completa ressecção tumoral. A paciente encontra-se bem 7 anos após a cirurgia com crescimento e desenvolvimento puberal normal, sem evidência de recidiva tumoral. São revistas as principais manifestações clínicas dos tumores ovarianos, sua classificação e o estadiamento dos tumores de estroma ovariano/cordão sexual, seu seguimento empregando diversos marcadores tumorais e hormonais. Finalmente é analisado o tratamento e prognóstico destes pacientes.

[Granulosa-stromal tumor of the ovary: a case of mixed germ cell-cord stromal tumor of the ovary with endocrinological considerations]. / Puberdade precoce associada a tumor misto ovariano (células germinativas- estroma-cordão sexual): aspectos clínicos, diagnósticos e manejo de um caso.

Granulosa-stromal tumors comprise 5 to 8% of all primary ovarian neoplasms. The first clinical manifestation is precocious puberty in most prepuberal patients. We report a case of mixed germ cell-cord stromal tumor of ovary in a 7.2 years old girl, who presented with isosexual pseudo-precocious puberty of progressive outcome. Serum testosterone, estradiol and 17alphaOH-progesterone levels were increased. Abdominal-pelvic ultrasound revealed a right ovarian mass. Unilateral salpingo-oophorectomy was performed with complete resection of the tumor. The patient is well 7 years after surgery with normal pubertal and growth development and no signs of tumor relapse. We review the clinical manifestations of ovarian tumors, classification and staging of sex cord-stromal tumors, follow-up, tumor markers, treatment and prognosis.

La afectación ganglionar en los carcinomas escamosos de pulmón.Interés de la proteína oncogénica erbB2 y el Cyfra 21.1 / Lymph nodes involvement from lung squamous carcinomas. Interest of erbB2 oncogenic protein and Cyfra 21.1

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