Carcinoma de Células Acinares de Pâncreas em Paciente de 9 anos: Relato de Caso / Acinar Cell Carcinoma of the Pancreas in a 9-Year-Old Patient: Case Report

O Carcinoma de Células Acinares (CCA) é uma neoplasia epitelial maligna, composta por células com semelhança morfológica com células acinares e com evidências de produção de enzimas exócrinas pelas células neoplásicas. CCA é um tumor extremamente raro e agressivo: representa cerca de 1% de todas as neoplasias pancreáticas, e a taxa de sobrevida global de 5 anos encontra-se inferior a 10%. Esse relato justifica-se não só pela rara ocorrência da doença, mas também pela escassa literatura acerca dos conhecimentos da patologia estudada. Além disso, o CCA é um tumor com difícil diagnóstico e extremamente agressivo; o tratamento dessa doença continua sendo aprofundado, uma vez que novos estudos sugerem a presença de micrometástases ­ indetectáveis em exames de imagem ­ em pacientes submetidos à ressecção cirúrgica total com margens negativas. Dessa forma, há a necessidade de incrementar relatos de caso à bibliografia existente. O presente trabalho trata-se de um relato de caso de Carcinoma de Células Acinares no pâncreas de paciente do sexo masculino de 9 anos diagnosticado no Hospital da Criança Santo Antônio do Complexo Hospitalar Santa Casa de Misericórdia de Porto Alegre.

Acinar Cell Carcinoma (ACC) is an epithelial malignant neoplasm composed of cells with morphological similarity to acinar cells and with evidence of exocrine enzyme production by the neoplastic cells. ACC is extremely rare and an aggressive tumor: it represents about 1% of all pancreatic neoplasms, and the overall 5-year survival rate is less than 10%. This report justifies itself by the rare occurrence of the disease and by the scarce literature concerning the pathology studied. Moreover, ACC is a tumor that is difficult to diagnose and extremely aggressive; the treatment of this disease continues under investigation since new studies suggest the presence of micrometastases - undetectable in imaging studies - in patients undergoing total surgical resection with negative margins. Thus, there is a need to add case reports to the existing bibliography. The present study is a case report of Acinar Cell Carcinoma of the pancreas in a 9-year-old male patient diagnosed at Santo Antônio Children's Hospital, Hospital Complex Santa Casa in Porto Alegre (Hospital da Criança Santo Antônio do Complexo Hospitalar Santa Casa de Misericórdia).

Síndrome de hipersecreción de lipasa y lesiones óseas en el cáncer de células acinares del páncreas / Lipase hypersecretion syndrome and bone lesions in acinar cell cancer of the pancreas. Report of one case

Lipase hypersecretion syndrome (LHS) is a rare paraneoplastic syndrome, associated with acinar cell carcinoma of the pancreas (ACCP) in 10% to 15% of patients. Clinically, LHS manifests itself with the appearance of subcutaneous fibrocystic nodules, associated with trophic changes in the overlying skin, such as ulcers or fistulas that are difficult to manage, mainly affecting the lower extremities. Additionally, lipolysis near the joints and in the intraosseous adipose tissue can cause bilateral arthralgias, especially of the knees and ankles. We report a 57-year-old man, with a history of insulin resistance and allergic rhinitis, who presented in June 2019 with multiple subcutaneous nodules in the lower extremities, predominantly in both ankles, associated with arthralgia in that region. Additionally, a CT scan of the abdomen revealed a significant abdominal mass, measuring approximately 17 cm and in contact with the body and tail of the pancreas, pathologically compatible with an ACCP. Treatment with capecitabine was started with a favorable progression. The patient currently presents a small left lateral retro malleolar fistula, which, given the analyzes, studies and reviewed literature is concluded to be a lesion in the context of LHS.

Carcinoma de células acinares de parótida: reporte de un caso y revisión de literatura / Acinic cell carcinoma of parotid: A case report and review of literature

Resumen Los tumores malignos de glándulas salivales (TMGS) constituyen un grupo infrecuente de cánceres de una gran variedad histológica. Dentro de las neoplasias de estirpe epitelial encontramos al carcinoma de células acinares (CCA), que representa entre un 6%-10% de todos los TMGS. No posee patrones clínicos ni radiológicos específicos, pero comparte características comunes con otros TMGS que serán discutidas a lo largo del reporte. Cabe destacar que en términos generales es un cáncer de bajo grado y poco recidivante, por lo que un tratamiento oportuno y un seguimiento estricto mejoran el pronóstico para este tipo de pacientes. Su diagnóstico se establece con evidencia histopatológica que confirme la presencia de diferenciación epitelial de tipo acinar. El tratamiento consiste en la exéresis tumoral con márgenes libres por parotidectomía suprafacial o total, asociado a vaciamiento cervical si se detecta compromiso nodal. Adicionalmente, se debe sugerir terapia adyuvante ante la presencia de un factor de mal pronóstico. Se presenta un caso y se realiza revisión de literatura.

Abstract Malignant salivary gland tumors (TMGS) constitute an infrequent group of cancers of a wide histological variety. Within the epithelial lineage neoplasms, we find acinar cell carcinoma that represent between 6%-10% of all TMGS. It does not have specific clinical or radiological patterns, but it shares common characteristics with other TMGS that will be discussed throughout the report. It should be noted that in general terms it is a low-grade cancer with low recurrence rates, so timely treatment and strict follow-up improve the prognosis for this type of patient. Its diagnosis is established with histopathological evidence that confirms the presence of acinar-type epithelial differentiation. Treatment consists of tumor excision with free margins by suprafacial or total parotidectomy, associated with cervical lymph node dissection if nodal locoregional metastasis is detected. Additionally, adjuvant therapy should be suggested in the presence of a poor prognostic factor. A case is presented and a literature review is carried out.

Carcinoma de células acinares: infrecuente tumor pancreático exocrino / Acinar cell carcinoma: a rare exocrine pancreatic tumor

RESUMEN El cáncer pancreático es aquel que comienza en el páncreas y constituye la neoplasia más letal que puede padecer un ser humano. Aunque las células más comunes en el páncreas son las células acinares, la transformación maligna de estas es infrecuente. El adenocarcinoma de células acinares es un tumor maligno muy raro del páncreas exocrino, con menos de un caso por millón de habitantes en los Estados Unidos, y representa menos del 1 % de las neoplasias pancreáticas primarias. Se presentó una paciente femenina de 64 años de edad operada de un carcinoma de células acinares del páncreas. Se revisó la literatura sobre esta temática y se insistió en que dado la topografía pancreática, las neoplasias de este tipo, se diagnostican frecuentemente en la fase final de la enfermedad; por lo que resulta imprescindible, bajo contextos similares, pensar en este tipo de cáncer como posibilidad diagnóstica.

ABSTRACT Pancreatic cancer is a cancer that begins in the pancreas and is the most lethal neoplasm that a human being can suffer from. Although the most common cells in the pancreas are acinar cells, their malignant transformation is rare. Acinar cell adenocarcinoma is a very rare malignant tumor of the exocrine pancreas, with less than one case per million population in the United States, accounting for less than 1% of primary pancreatic neoplasms. We present a 64-year-old female patient operated on for acinar cell carcinoma of the pancreas. The literature on this topic was reviewed, emphasizing on this type of neoplasm, which is frequently diagnosed in the final stage of the disease given its pancreatic topography; that is why, under similar contexts, thinking about this type of cancer as a diagnostic possibility is essential.

Primary nonlymphoid gastrointestinal neoplasms in dogs in Rio Grande do Sul / Neoplasmas gastrointestinais primários não linfoides em cães no Rio Grande do Sul

Gastrointestinal neoplasms (GIN) are uncommon in dogs, but they mainly show malignant behavior and poor prognosis. The types of GIN in dogs and their frequency, as well as their epidemiological and histopathological characteristics were analyzed through a retrospective study of biopsies from 24.711 dogs from 2005 to 2017. Additionally, histological sections of neoplasms were subjected to immunohistochemistry (IHC) using antibodies against pancytokeratin, vimentin, smooth muscle actin, c-Kit, S-100, CD31, CD79αcy, and neuron-specific enolase. Of the total samples from dogs analyzed, 88 corresponded to GIN. Neoplasms occurred more frequently in purebred dogs (64.8%, 57/88), males (53.4%, 47/88), with a median age of 10 years. The intestine was affected by 84.1% (74/88) of the cases. Of these, the large intestine was the most affected (67.6%, 50/74). Most of the neoplasms had malignant behavior (88.6%, 78/88). Regarding the classification of neoplasms, 46.6% (41/88) of the diagnoses corresponded to epithelial, 46.6% (41/88) were mesenchymal, 5.7% (5/88) were hematopoietic, and 1.1% (1/88) was neuroendocrine. The most frequently diagnosed neoplasms were papillary adenocarcinoma (19.3%, 17/88), leiomyosarcoma (17.0%, 15/88), gastrointestinal stromal tumors (GISTs) (12.5%, 11/88), and leiomyoma (5.0%, 8/88). Adenocarcinomas were located mainly in the rectum, whereas leiomyosarcomas and GISTs developed mainly in the cecum. Epithelial neoplasms showed a greater potential for lymphatic invasion whereas mesenchymal neoplasms appeared to be more expansive with intratumoral necrosis and hemorrhage. Immunohistochemistry was found to be an important diagnostic technique for the identification of infiltrating cells in carcinomas and an indispensable technique for the definitive diagnosis of sarcomas.(AU)

Neoplasmas gastrointestinais (NGI) são pouco comuns em cães, mas possuem principalmente comportamento maligno e prognóstico reservado. Os tipos de NGI em cães e sua frequência, bem como características epidemiológicas e histopatológicas foram analisados por meio de um estudo retrospectivo dos exames de biópsias de 24.711 cães entre os anos de 2005 a 2017. Adicionalmente, cortes histológicos de NGI foram submetidos à técnica de imuno-histoquímica (IHQ), utilizando os anticorpos anti-pancitoqueratina, vimentina, actina de músculo liso, c-Kit, S-100, CD31, CD79αcy e enolase neurônio específica. Do total de cães analisados, 88 corresponderam a NGI não linfoides. Os neoplasmas ocorreram com maior frequência em cães de raça pura (64,8%, 57/88), machos (53,4%, 47/88), com mediana de idade de 10 anos. O intestino foi acometido em 84,1% dos casos (74/88). Destes, o intestino grosso foi o segmento mais afetado (67,6%, 50/74). A maior parte dos neoplasmas tinha comportamento maligno (88,6%, 78/88). Quanto à classificação, 46,6% (41/88) dos diagnósticos corresponderam a neoplasmas epiteliais, 46,6% (41/88) mesenquimais, 5,7% (5/88) hematopoiéticos e 1,1% (1/88), neuroendócrino. Os neoplasmas mais frequentemente diagnosticados foram adenocarcinoma papilar (19,3%, 17/88), leiomiossarcoma (17,0%, 15/88), tumor estromal gastrointestinal (GIST) (12,5%, 11/88) e leiomioma (12,5%, 8/88). Adenocarcinomas localizavam-se principalmente no reto, enquanto leiomiossarcoma e GISTs desenvolveram-se principalmente no ceco. Os neoplasmas epiteliais demonstraram um potencial maior de invasão linfática enquanto que os mesenquimais aparentaram ser mais expansivos, com necrose e hemorragia intratumorais. A imuno-histoquímica mostrou ser uma técnica diagnóstica importante para a identificação de células neoplásicas infiltravas no caso dos carcinomas e uma técnica indispensável para o diagnóstico definitivo de sarcomas.(AU)

Pancreatitis, Panniculitis, and Polyarthritis Syndrome Simulating Cellulitis and Gouty Arthritis / 대한소화기병학회잡지

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but critical disease with a high mortality rate. The diagnostic dilemma of PPP syndrome is the fact that symptoms occur unexpectedly. A 48-year-old man presented with fever and painful swelling of the left foot that was initially mistaken for cellulitis and gouty arthritis. The diagnosis of PPP syndrome was made based on the abdominal CT findings and elevated pancreatic enzyme levels, lobular panniculitis with ghost cells on a skin biopsy, and polyarthritis on a bone scan. The pancreatitis and panniculitis disappeared spontaneously over time, but the polyarthritis followed its own course despite the use of anti-inflammatory agents. In addition to this case, 30 cases of PPP syndrome in the English literature were reviewed. Most of the patients had initial symptoms other than abdominal pain, leading to misdiagnosis. About one-third of them were finally diagnosed with a pancreatic tumor, of which pancreatic acinar cell carcinoma was the most dominant. They showed a mortality rate of 32.3%, associated mainly with the pancreatic malignancy. Therefore, PPP syndrome should be considered when cutaneous or osteoarticular manifestations occur in patients with pancreatitis. Active investigation and continued observations are needed for patients suspected of PPP syndrome.

Pancreatitis, Panniculitis, and Polyarthritis Syndrome Simulating Cellulitis and Gouty Arthritis / 대한소화기학회지

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but critical disease with a high mortality rate. The diagnostic dilemma of PPP syndrome is the fact that symptoms occur unexpectedly. A 48-year-old man presented with fever and painful swelling of the left foot that was initially mistaken for cellulitis and gouty arthritis. The diagnosis of PPP syndrome was made based on the abdominal CT findings and elevated pancreatic enzyme levels, lobular panniculitis with ghost cells on a skin biopsy, and polyarthritis on a bone scan. The pancreatitis and panniculitis disappeared spontaneously over time, but the polyarthritis followed its own course despite the use of anti-inflammatory agents. In addition to this case, 30 cases of PPP syndrome in the English literature were reviewed. Most of the patients had initial symptoms other than abdominal pain, leading to misdiagnosis. About one-third of them were finally diagnosed with a pancreatic tumor, of which pancreatic acinar cell carcinoma was the most dominant. They showed a mortality rate of 32.3%, associated mainly with the pancreatic malignancy. Therefore, PPP syndrome should be considered when cutaneous or osteoarticular manifestations occur in patients with pancreatitis. Active investigation and continued observations are needed for patients suspected of PPP syndrome.

Prognostic utility of epidermal growth factor receptor (EGFR) expression in prostatic acinar adenocarcinoma

Background: Epidermal growth factor receptor (EGFR) is potential prognostic biomarker expressed in many human cancers. Prognostic significance of EGFR immunohistochemical expression has not been established in prostatic acinar adenocarcinoma, therefore we aimed to evaluate the frequency of expression of EGFR in prostatic adenocarcinoma and its association with other prognostic parameters. Methods: The study included 123 cases of biopsy proven prostatic acinar adenocarcinoma treated at Liaquat National hospital, Karachi from January 2013 till December 2017. Paraffin blocks of all cases were retrieved; sections were cut and stained with haematoxylin and eosin. Pathologic characteristics including tumor quantification, WHO grade group, gleason score, perineural and lymphovascular invasion were evaluated. EGFR immunohistochemistry (IHC) was performed on all tissue blocks. Results: Mean age of the patients included in the study was 69.05±8.68years. High gleason scores i.e. 8 & 9 were noted in 22% (27 cases) and 22.8% (28 cases) respectively. Similarly, 22.8% (28 cases) showed WHO grade group 5. 52.8% (65 cases) had > 50% tissue involvement by carcinoma and perineural invasion was seen in 37.4% (46 cases). Positive EGFR expression was noted in 18.7% (23 cases), while 81.3% (100 cases) showed negative EGFR expression. Significant association of EGFR expression was noted with gleason score (p-value = < 0.001), WHO grade (p = < 0.001), tumor quantification (p =0.007) and perineural invasion (p = < 0.001). Moreover, significant association of EGFR expression was also seen with disease recurrence and Her2neu over expression. Patients with low gleason scores (score 6 and 7) and lower grade group (1, 2 & 3) were less likely to have positive EGFR expression as compared to patients with high gleason score (score 9) and higher grade group (5). Similarly, patients with perineural invasion were more likely to have positive EGFR expression. Conclusion: We found a relatively low EGFR expression in our patients with prostatic adenocarcinoma; however, its association with poor prognostic parameters like high gleason score, higher grade group, perineural invasion, higher tissue involvement by cancer and disease recurrence signifies its importance as a prognostic parameter in prostatic acinar adenocarcinoma (AU)

Cáncer de páncreas en edad pediátrica / Pancreatic cancer in pediatric age

Introducción: Los tumores malignos pancreáticos en pediatría son extremadamente infrecuentes. La sobrevida en el cáncer pancreático a cinco años es baja. Objetivo: Informar a la comunidad médica acerca de una variante poco frecuente de tumor maligno pancreático en edad pediátrica. Presentación del caso: Paciente masculino de 17 años de edad, de la raza negra, que asiste a consulta en julio de 2017 por dolor en hemiabdomen superior, se considera una gastritis y se medica con dieta y antiácidos. Posteriormente comienza con dolor abdominal recurrente, pérdida de peso, anorexia, dispepsias, ictericia en piel y mucosas. Acude al gastroenterólogo quien indica una endoscopia digestiva alta y realiza el diagnóstico del tumor mediante biopsia endoscópica transduodenal. Se opera y reseca gran tumor de cabeza del páncreas junto con primera, segunda y tercera porción del duodeno (pancreatoduodenectomía). El tumor en conjunto midió aproximadamente 15 X 20 cm de diámetro y fue una cirugía completa sin lesión microscópica residual. El resultado de la biopsia indicó que se trataba de un adenocarcinoma acinar del páncreas pobremente diferenciado. Conclusión: Existen pocos casos publicados en la edad pediátrica con esta variante de tumor pancreático. Se documenta la importancia de la cirugía en la cura de la enfermedad(AU)

Introduction: Pancreatic malignancies in pediatrics are extremely infrequent, among them ductal adenocarcinoma and acinar adenocarcinoma. Survival in pancreatic cancer at five years is low. Objective: To inform the medical community about an uncommon variant of pancreatic malignant tumor in pediatric age. Case presentation: Male patient of 17 yesar of age, of the black race, who attended consultation in July of 2017 for pain in upper abdomen, is considered a gastritis and is medicated with diet and antacids. Subsequently begins with recurrent abdominal pain, weight loss, anorexia, dyspepsia, and skin and mucous. Go to the gastroenterologist who indicates an upper gastrointestinal endoscopy and perform the diagnosis of the tumor by transduodenal endoscopic biopsy. A large head tumor of the pancreas is operated on and resected together with the first, second and third portion of the duodenum (pancreatoduodenectomy). The tumor as a whole measured approximately 15 X 20 cm in diameter and was a complete surgery without residual microscopic lesion. The result of the biopsy indicated that it was an acinar adenocarcinoma of the poorly differentiated pancreas. Conclusion: There are few cases published in the pediatric age with this variant of pancreatic tumor. The importance of surgery in the cure of the disease is documented(AU)

Incidental Discovery of Acinic Cell Carcinoma of the Oral Mucosa with Metachronous Adenoid Cystic Carcinoma of the Upper Lip / Hallazgo Incidental de Carcinoma de Células Acinares de la Mucosa Oral con Carcinoma Adenoide Quístico Metacrónico del Labio Superior

ABSTRACT: Multiple salivary gland tumors represent an unusual event characterized by the development of composite lesions originated from minor or major salivary glands. These neoplasms can be categorized into three perspectives: Histologic type, time of appearance and topographic distribution. We report an unusual case of a 73-year-old black man with an acinic cell carcinoma (ACC) of the oral mucosa discovered incidentally during surgical removal of an adjacent mucocele. Approximately one year after the first consultation, the patient was seen at the local cancer reference center with a third lesion that was diagnosed as an adenoid cystic carcinoma (AdCC) of the upper lip. The patient underwent surgical reconstruction of the treated areas and has been free of the disease for the past year. To our knowledge, the combination of ACC and AdCC in intraoral sites has not been reported in the literature.

RESUMEN: Los tumores de glándulas salivales múltiples representan un evento inusual caracterizado por el desarrollo de lesiones compuestas, originadas en glándulas salivales menores o mayores. Estos neoplasmas se pueden categorizar en tres perspectivas: tipo histológico, tiempo de aparición y distribución topográfica. Reportamos un caso inusual de un hombre negro de 73 años con un carcinoma de célula acínica (ACC) de la mucosa oral descubierta incidentalmente durante la extirpación quirúrgica de un mucocele adyacente. Aproximadamente un año después de la primera consulta, el paciente se presentó en el centro de referencia del cáncer local con una tercera lesión que fue diagnosticada como carcinoma adenoide quístico (AdCC) del labio superior. El paciente se sometió a la reconstrucción quirúrgica de las áreas tratadas y durante el último año no ha presentado recurrencia de la enfermedad. De acuerdo a nuestro conocimiento la combinación de ACC y AdCC en sitios intraorales no se ha informado en la literatura.

Primary acinic cell carcinoma in mandible: a case report / 华西口腔医学杂志

Acinic cell carcinoma is a relatively rare salivary gland tumor predominantly occurring in the major glands. Therefore, acinic cell carcinoma rarely occurs in the mandible. In this study, a case of primary acinic cell carcinoma of the mandible was reported, and relevant literature was reviewed. The etiology, clinical symptom, image and histological features, diagnosis, treatment, and prognosis of acinic cell carcinoma in the mandible were discussed.

Carcinoma de células acinares de glándula lacrimal metastásico en columna torácica. Reporte de un caso y revisión de la literatura / Acinar cell carcinoma of lacrimal gland metastatic a thoracic spine. Case report and literature review

Resumen El carcinoma de células acinares es una neoplasia poco frecuente que se presenta principalmente en las glándulas salivales. Presentamos el caso de un paciente femenino de 48 años con dolor, paresia palpebral derecha y aumento de volumen. Biopsia que confirma diagnóstico, manejada con exenteración orbitaria derecha más RT. Durante seguimiento seis años después se presenta dolor columna dorsal, RMN con lesión osteoblástica en T2 biopsia con metástasis de carcinoma de células acinares. Debido a su baja incidencia el comportamiento del carcinoma de células acinares de la glán dula lacrimal es incierto, no hay reportes en la literatura de lesiones metastásicas únicas en columna.

Abstract Acinar cell carcinoma is a rare neoplasm occurs primarily in the salivary glands. We report the case of a female patient of 48 years with pain, right palpebral paresis, and increased volume. Biopsy confirmed diagnosis, handled right exenteration more RT. During follow-up six years after dorsal spine pain, MRI with T2 lesion biopsy osteoblastic metastatic carcinoma of acinar cells. Because of its low incidence behavior acinar cell carcinoma of the lacrimal gland is uncertain, there are no reports in the literature of metastatic lesions unique column.

Efficacy of Chemotherapy in Patients with Unresectable or Metastatic Pancreatic Acinar Cell Carcinoma: Potentially Improved Efficacy with Oxaliplatin-Containing Regimen / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Pancreatic acinar cell carcinoma (ACC) is a rare cancer of the exocrine pancreas. Because of its rare incidence, the efficacy of chemotherapy in this patient population has been largely unknown. Therefore, we retrospectively analyzed the outcomes of patients with advanced pancreatic ACC who received chemotherapy. MATERIALS AND METHODS: Between January 1997 and March 2015, 15 patients with unresectable or metastatic pancreatic ACC who received systemic chemotherapy were identified in Asan Medical Center, Korea. RESULTS: The median age was 58 years. Eleven and four patients had recurrent/metastatic and locally advanced unresectable disease. The median overall survival in all patients was 20.9 months (95% confidence interval [CI], 15.7 to 26.1). As first-line therapy, intravenous 5-fluorouracil were administered in four patients (27%), gemcitabine in five (33%), gemcitabine plus capecitabine in two (13%), oxaliplatin plus 5-fluorouracil/leucovorin (FOLFOX) in two (13%), and concurrent chemoradiotherapy followed by capecitabine maintenance therapy in two (13%). The objective response rate (ORR) to chemotherapy alone was 23% and the median progression-free survival (PFS) was 5.6 months (95% CI, 2.8 to 8.4). After progression, second-line chemotherapy was administered in eight patients, while four patients received FOLFOX and the other four patients received gemcitabine. The ORR was 38%, and patients administered FOLFOX had significantly better PFS than those administered gemcitabine (median, 6.5 months vs. 1.4 months; p=0.007). The ratio of time to tumor progression (TTP) during first-line chemotherapy to TTP at second-line chemotherapy was significantly higher in patients administered FOLFOX (4.07; range, 0.87 to 8.30) than in those administered gemcitabine (0.12; range, 0.08 to 0.25; p=0.029). CONCLUSION: Our results suggest that oxaliplatin-containing regimens may have improved activity against pancreatic ACC.

Efficacy of Chemotherapy in Patients with Unresectable or Metastatic Pancreatic Acinar Cell Carcinoma: Potentially Improved Efficacy with Oxaliplatin-Containing Regimen / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Pancreatic acinar cell carcinoma (ACC) is a rare cancer of the exocrine pancreas. Because of its rare incidence, the efficacy of chemotherapy in this patient population has been largely unknown. Therefore, we retrospectively analyzed the outcomes of patients with advanced pancreatic ACC who received chemotherapy. MATERIALS AND METHODS: Between January 1997 and March 2015, 15 patients with unresectable or metastatic pancreatic ACC who received systemic chemotherapy were identified in Asan Medical Center, Korea. RESULTS: The median age was 58 years. Eleven and four patients had recurrent/metastatic and locally advanced unresectable disease. The median overall survival in all patients was 20.9 months (95% confidence interval [CI], 15.7 to 26.1). As first-line therapy, intravenous 5-fluorouracil were administered in four patients (27%), gemcitabine in five (33%), gemcitabine plus capecitabine in two (13%), oxaliplatin plus 5-fluorouracil/leucovorin (FOLFOX) in two (13%), and concurrent chemoradiotherapy followed by capecitabine maintenance therapy in two (13%). The objective response rate (ORR) to chemotherapy alone was 23% and the median progression-free survival (PFS) was 5.6 months (95% CI, 2.8 to 8.4). After progression, second-line chemotherapy was administered in eight patients, while four patients received FOLFOX and the other four patients received gemcitabine. The ORR was 38%, and patients administered FOLFOX had significantly better PFS than those administered gemcitabine (median, 6.5 months vs. 1.4 months; p=0.007). The ratio of time to tumor progression (TTP) during first-line chemotherapy to TTP at second-line chemotherapy was significantly higher in patients administered FOLFOX (4.07; range, 0.87 to 8.30) than in those administered gemcitabine (0.12; range, 0.08 to 0.25; p=0.029). CONCLUSION: Our results suggest that oxaliplatin-containing regimens may have improved activity against pancreatic ACC.

A Pediatric Case of Mixed Acinar-Neuroendocrine Carcinoma / 소아외과

Mixed acinar-neuroendocrine carcinoma (MANEC) is a malignant pancreatic tumor that rarely occurs in children. It is diagnosed pathologically according to the proportion of neuroendocrine cells present, highlighting the need for surgical biopsy. A 13-year-old boy presented with a 10-cm palpable mass on CT. Surgical resection was performed, and the pathological diagnosis was MANEC. There were no postoperative complications, and the patient was discharged from the hospital 10 days after surgery. He is presently undergoing adjuvant chemotherapy. We reviewed historical MANEC cases published in the English literature. We concluded that pathological analysis of a surgically resected specimen is necessary for an accurate diagnosis of MANEC, and that publication of more cases is needed to determine the optimal management strategy for MANEC.

Intraductal Carcinoma of the Prostate Gland: Recent Advances

Intraductal carcinoma of the prostate (IDC-P) is characterized by prostatic carcinoma involving ducts and/or acini. The presence of IDC-P is usually associated with a high-grade Gleason score, large tumor volume, and adverse prognostic parameters, including extraprostatic extension and seminal vesicle invasion. When present, IDC-P is associated with worse outcomes, regardless of treatment status. IDC-P is included in a broader diagnostic category of atypical cribriform lesions of the prostate gland. This category of lesions also includes high-grade prostatic intraepithelial neoplasia (HGPIN), urothelial carcinoma involving prostatic ducts or acini, and prostatic ductal adenocarcinoma, amongst other intraductal proliferations. Differentiating between these entities is important as they have differing therapeutic and prognostic implications for patients, although differential diagnosis thereof is not always straightforward. The present review discusses IDC-P in regards to its morphological characteristics, molecular features, and clinical outcomes. Given the current state of knowledge, the presence of IDC-P should be evaluated and documented correctly in both radical prostatectomy and needle biopsy specimens, and the clinical implications thereof should be taken into consideration during treatment and follow up.

Nasal endoscope surgery of acinic cell carcinoma of salivary gland on nasal septum: a case report / 临床耳鼻咽喉头颈外科杂志

A 67-year-old male patient was admitted because of "the right side nasal obstruction repeatedly for 4 years". He got nasal obstruction 4 years ago, especially for the right side nasal cavity, sometimes got blood in his nasal discharge, then the symptom relieved after accepting treatment in local hospital. During the 4 years, the symptom repeatedly occurrence. Three days before hospitalization, the CT examination indicated abnormal things in his nasal cavity and the bone of his nasal sinus had been destroyed. Some abnormal organism were sent to pathological examination, and the report indicated it is acinic cell carcinoma of salivary gland. During the nasal endoscope surgery, a red goiter was found in his nose with its surface crude and brittle. Then we cut the goiter by nasal endoscope, during the operation we find the bottom of the goiter is on the nasal septum. Two weeks after the operation, the patient received the radiation therapy. One year after the operation he doesn't get the abnormal symptom and the nasal MRI not found recidivation.

Polyarthritis and Pancreatic Panniculitis in a Patient with Pancreatic Acinar Cell Carcinoma / 대한피부과학회지

No abstract available.

A Case of Acinic Cell Carcinoma Involving Skin

No abstract available.