Neoplasia blástica de células dendríticas plasmocitoides: reporte de un caso y revisión de la literatura / Plasmacytoid dendritic cell blast neoplasia: case report and literature review

La neoplasia de células blásticas plasmocitoides dendríticas esun linfoma cutáneo poco frecuente y de mal pronóstico, que característicamenteexpresa antígenos CD4 y CD56. Clínicamentepresenta placas o nódulos de coloración violácea, únicos o múltiples.El diagnóstico se confirma con el estudio anatomopatológicoque evidencia células linfoblástica y fenotipo CD4+ y CD56+, conausencia de marcadores para células mieloides, linfoides B y T ycélulas NK. Presentamos el reporte de un caso de un paciente desexo masculino de 65 años de edad y una revisión de la literatura.

Blastic plasmacytoid dendritic cell neoplasm is a rare andhighly aggressive cutaneous lymphoma that characteristicallyexpress CD4 and CD56 antigens. Clinically presents violet colouredplaques or nodules, in a unique or multiple presentation.The diagnosis is confirmed with the anatomophatologic studythat evidence linfoblastic cells with CD4+ and CD56+ phenotype,and no mieloids, linfoids B and T and natural killers cellsmarkers. We present a case report of a 65-years-old male anda literature review.

Enfermedad de Castleman, un diagnóstico diferencial que no se debe olvidar en los tumores de mediastino: Caso clínico / Mediastinal Castleman's disease: Report of one case

Castleman's disease is an uncommon lymphoproliferative disorder which can be unicentric or multicentric. Hialine vascular variant is the most common pathologic form, which is usually unicentric and presenting as mediastinal tumors. We report a 31-year-old female with a history of retrosternal pain. A chest CAT sean showed a tumor in the posterior mediastinum. The patient was operated and the tumor excised. The pathology report showed a Castleman's disease. No other tumors were found in the patient, who had a favorable evolution.

Quiste broncogénico de localización poco habitual: reporte de 2 casos y revisión de la literatura / Bronchogenic cyst of unusual location: report of two cases and review of the literature

El quiste broncogénico es un remanente del desarrollo de traquea y bronquios. Alteraciones en la migración de células durante el desarrollo, permite que estos grupos originen quistes revestidos por epitelio respiratorio. La ubicación más habitual es la intratoráxica, dentro de ella, la intrapulmonar es la más frecuente, seguida de la mediastínica. El diagnóstico definitivo se establece mediante la biopsia diferida. Es una patología poco frecuente y las presentaciones extra torácicas son muy infrecuentes, por lo que compartimos dos casos, uno de ubicación sublingual y otro de ubicación subcutánea en zona escapular.

The bronchogenic cyst is a remanent of the development of the tracheobronchial tree. Alterations in cell migration during development cause the formation of cyst lining by respiratory epithelium. They are usually located in the thorax in lung and mediastinum. The definite diagnosis is realized with the pathology report. This is an uncommon pathological condition and the extra- thoracic forms are very unusual. We present two cases, one of which is of sublingual location and the other of subcutaneous location at the scapular site.

Cáncer gástrico sin compromiso ganglionar linfático (pN0): características demográfico-patológicas y factores pronósticos / Prognostic factors for gastric cancer without lymph node involvement

Background: The absence of lymph node involvement (N0) in gastric cancer is associated with a better survival. However some N0 gastric tumors still have a bad prognosis. Aim: To study demographic and morphological variables associated with prognosis in N0 gastric carcinoma. Material and Methods: Review of pathologica records of a regional general hospital, identifying patients with a N0 gastric cancer surgically excised between 1986 and 2003. Results: In the study period, 459 gastrectomies were performed for gastric cancer and in 32 percent, the tumor was devoid of lymph node involvement. These later patients were followed for a median of 64 months with a 71 percent fve years actuarial survival. Bivariate analysis identifed age, tumor size, gastric wallinfiltration, pathological type according to Lauren and Ming, lymphovascular involvement, number of lymph nodes excised and TNM stage as prognostic values Multivariate analysis disclosed the level of gastric wallinfiltration, the presence of a poorly differentiated tumor, lymphatic vascular involvement, number of excise lymph nodes and tumor size as independent prognostic factors. Conclusions: N0 gastric tumors are found in 32 percent of gastrectomies for gastric cancer and have a 71 percent fve years actuarial survival. Gastric wallinfiltration, pathological degree of differentiation tumor size and lymphovascular involvement are independent prognostic factors.

Puntaje de Gleason en cáncer de próstata: Correlación de la biopsia por punción y prostatectomía radical / Concordance between Gleason scores of trans rectal biopsies and the surgical piece of radical prostatectomy

Background: Gleason pathological score in prostate cancer is an importantprognostic indicator. However, the concordance between the score of trans rectal needle biopsies and the final score of the surgical piece may be variable. Aim: To analyze the concordance between Gleason scores of trans rectal prostate biopsies and those of the surgical piece obtained after prostatectomy. Material and Methods: Retrospective analysis of 168 pathological records of radical prostatectomies, performed between 1993 and 2009. All these patients had also a trans rectal biopsy performed previously. Patients with less than 12 tissue cylinders obtained during the trans rectal biopsy or incomplete data were not included in this analysis. Results: Sixty eight percent of trans rectal biopsies had Gleason scores that were concordant with those of the surgical piece. The score was higher or lower in 27 and 10 percent of biopsies, respectively. Conclusions: Gleason scores of trans rectal biopsies and those of the surgical piece were concordant in 68 percent of cases in this series of pathological records.

Hidatidosis ósea: Reporte de 5 casos y revisión de la literatura / Bone hydatidosis: Report of five patients

Bone location of hydatid cysts occurs in 0.5 to 3 percent of all cases of hydatidosis. The most common bones involved are spine, long bones and pelvis. We report five patients with bone hydatidosis. A 24-year-old male consulting for paraparesis and numbness of lower limbs; CAT scan showed hydatid cysts located in D2, D3 and D4 vertebral bodies. A 47-year-old male consulting for a fracture of the femur; X rays disclosed multiple hydatid cysts in the femur. A 13-year-old female consulting for claudication of the right lower limb; X ray examination showed a hydatid cyst in the iliac bone. A 21-year-old women presenting with weakness of the lower limbs and voiding problems; CAT scan showed a vertebral hydatidosis and spinal cord compression. A 67-year-old female presenting with weakness of the lower limb and loss of sphincter control; CAT scan showed hydatid cysts in D4 vertebral body.

Evaluación de factores histológicos pronósticos de sobrevida en melanoma maligno cutáneo, seguimiento de 13 años / Prognostic factors for survival of malignant melanomas

Background: The incidence of malignant melanoma is increasing steadily. Aim: To study pathological prognostic factors in malignant melanomas. Material and Methods: Review of pathological records of cutaneous malignant melanomas diagnosed between 1995 and 2008. Medical records of patients were re-viewed and those with distant metastases at the moment of surgery were excluded. Follow up was performed contacting patients and reviewing death records at the Chilean National Identification Registry. The cause of death was classified as related to the melanoma or unrelated. Results: One hundred sixty two records, corresponding to patients aged from 13 to 93 years (51 percent women), were included in the study. Twenty nine percent of patients died during the follow up period that ranged from 1 to 127 months. Five and ten year's survival was 71 and 60 percent respectively. A multivariable Cox analysis demonstrated that only the TNM classification of the primary tumor, which is categorized using the Breslow maximal depth, had prognostic significance. Compared to women, men had a 1.97 higher relative risk of dying due to the melanoma. Conclusions: TNM classification of melanomas is the most important prognostic factor for survival.

Introducción: El Melanoma Maligno (MM) de la piel ha aumentado su incidencia en 5 por ciento anual, se estima que 1 de cada 55 hombres y 1 de cada 77 mujeres desarrollará la enfermedad durante su vida. Método y Diseño: Estudio de cohorte retrospectivo de seguimiento de casos de melanoma maligno primario de la piel diagnosticados en la Unidad de Anatomía Patológica del Hospital Dr. Hernán Henríquez Aravena de Temuco. Se incluyeron 162 casos de MM primario de piel. El período de seguimiento fue de 1 a 164 meses. El análisis estadístico se realizó según método de Kaplan-Meier para la descripción de sobrevida según las variables de interés, utilizando la prueba no paramétrica Log-rank, modelo univariado y multivariado de Cox. Resultados: Se incluyeron 162 casos en la cohorte de estudio. El 28,7 por ciento de los pacientes falleció producto del melanoma durante el tiempo de observación (rango 1 a 127 meses, mediana 23 meses). La sobrevida general de la cohorte a 5 y 10 años fue de 71 por ciento y 60 por ciento respectivamente. El análisis multivariado de Cox demostró que sólo la clasificación del tumor primario según TNM es significativa. Los hombres tienen un riesgo relativo de 1,97 para morir por MM. La edad y distribución topográfica no resultaron ser variables pronosticas. Conclusión: En nuestro estudio se confirman los indicadores histológicos de pronóstico de sobrevida en población de la región de La Araucanía, los factores de mayor importancia son el espesor máximo según Breslow, presencia de úlcera, tipo histológico y nivel de infiltración de Clark.

Cáncer de colon y recto: descripción morfológica y clínica de 322 casos / Colon and rectum cancer: morphological and clinical description of 322 cases

El cáncer de colon y recto (CCR) es actualmente la cuarta causa de muerte por cáncer en Chile. Su incidencia, sin embargo, está aumentando continuamente en nuestra población. El objetivo de este estudio es describir aspectos morfológicos y clínicos de pacientes resecados por CCR. Estudio de cohorte retrospectiva. Se estudiaron 322 pacientes intervenidos por CCR entre 1987 y 2003 en el Hospital Hernán Henríquez Aravena de Temuco. Las variables clínicas y morfológicas estudiadas (todas ellas analizadas para los subgrupos de sujetos con tumores de colon y de recto) fueron edad, género, localización tumoral, forma y tamaño tumoral, nivel de infiltración, tipo histológico, grado de diferenciación histológico y compromiso tumoral de nodos linfáticos. Se utilizaron estadísticas descriptivas y analíticas; aplicando chi-cuadrado de Pearson y exacto de Fisher para las variables categóricas; y, T-test para variables continuas. La mediana de edad fue de 66 años, con promedio de edad para tumores de colon derecho (CD), transverso (CT), izquierdo (CI) y recto fue 62,2, 64,6, 64, y 64,4 años respectivamente (p=0,53). En CD e CI se verificaron 57 por ciento y 47 por ciento de mujeres respectivamente (p<0,05). El 69 por ciento de los casos correspondió a tumores de colon (24 por ciento CD, 4 por ciento CT y 41 por ciento CI) y 31 por ciento a tumores de recto. El tamaño tumoral promedio fue 67,2 +/- 33,1 mm CD, 53,5 +/- 19,7 mm CT, 44,1 +/- 22,3 mm. CI y 41,5 +/- 17,5 mm en recto (p<0,001). En CD la forma tipo Bormann I se observó en el 57 por ciento mientras que en CI lesiones anulares y ulceradas en 45 por ciento en CI. Se encontró 75 por ciento de tumores moderadamente diferenciados; correspondiendo el 82 por ciento a adenocarcinomas, 16 por ciento adenocarcinoma mucinoso y 2 por ciento carcinoma de células en anillo de sello. El 76 por ciento correspondió a tumores T3 y T4. Se encontró compromiso tumoral de nodos linfáticos en 39 por ciento de los cuales el 95 por ciento...

The colorectal cancer (CRC) is currently the fourth cause of cancer death in Chile. Its incidence, however, is continuously increasing in our population. The aim of this study is to describe morphological and clinical aspects of patients resected CRC. Retrospective cohort study. We studied 322 patients operated on for CRC between 1987 and 2003 in the Hernan Henriquez Aravena Hospital of Temuco. The clinical and morphological variables studied (all analyzed for subgroups of subjects with tumors of the colon and rectum) were age, gender, tumor location, tumor size and shape, level of infiltration, histological type, histological differentiation grade and tumor involvement lymph node. We used descriptive statistics and analytical, using Pearson chi-square and Fisher exact tests for categorical variables and T-test for continuous variables. The median age was66 years, with average age of the right colon tumors (RC), transverse (TC), left (LC) and rectum was 62.2, 64.6, 64, and 64.4 years respectively (p = 0.53). In RC and LC were observed 57 percent and 47 percent of women, respectively (p <0.05). 69 percent of the cases corresponded to tumors of the colon (24 percent RC, 4 percent TC and 41 percent LC) and 31 percent to cancer of the rectum. The average tumor size was 67.2 +/- 33.1 mm RC, 53.5 +/- 19.7 mm. TC, 44.1 +/- 22.3 mm LC and 41.5 +/- 17.5 mm in the rectum (p <0.001). On RC as Bormann type I was observed in 57 percent while the LC annular and ulcerative lesions 45 percent CI. We found 75 percent of moderately differentiated tumors, corresponding to 82 percent adenocarcinomas, 16 percent and 2 percent mucinous adenocarcinoma cell carcinoma signet-ring. 76 percent were T3 and T4 tumors. We found lymph node tumor involvement in 39 percent of which 95 percent were T3-T4 tumors. We checked at the regional level for patients with CRC clinical and morphological variables described in the literature.

Tumor de células de la granulosa del testículo, tipo juvenil: reporte de un caso y revisión de la literatura / Granulosa cell tumor of testis, juvenile type: case report and review of literature

El Tumor de Células de la Granulosa Juvenil (TCGJ) del testículo es una entidad clínico-patológica poco frecuente, que típicamente se presenta en la edad pediátrica representando el 15 por ciento de los tumores del estroma gonadal. Su comportamiento biológico es considerado benigno y la orquiectomía considerada curativa en estos pacientes. Se presenta el caso de un lactante de sexo masculino de 3 meses de edad con un TCGJ del testículo diagnosticado en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez Aravena de Temuco.

Juvenile granulosa cell tumor of the testis (JGCT) is a uncommon clinical and pathological entity, typically present in children accounting for 15 percent of gonadal stromal tumors. Its biological behavior is deemed benign and orchiectomy considered curative in these patients. We present a case of a male infant 3 months of age with a JGCT diagnosed in the Pathology Unit of Hospital Hernán Henríquez Aravena, Temuco.

Validez diagnóstica de la biopsia intraoperatoria en cirugía de lesiones mamarias palpables / Diagnostic value of frozen section biopsy during surgery for breast lesions or neoplasms

Background: During the surgical treatment of breast neoplasms (benign or malignant), frozen section biopsy is frequently requested to assess the kind of lesion and determine the surgical margins. Aim: To assess the diagnostic yield of frozen section breast biopsy. Material and methods: AH the pathological reports of frozen section biopsies and definitive biopsies of 337 women aged 26 to 88 years, operated for suspected breast neoplasms between 2002 and 2006, were reviewed. The sensitivity, specificity and predictive value of frozen section biopsy, were calculated using the definitive biopsy as the gold standard. Results: The definitive biopsy confirmed the presence of cancer in 290 women (86 percent). There were two false negative (0.59 percent) and no false positive frozen section biopsies for cancer detection. The sensibility for cancer detection was 99.3 percent and the specificity 100 percent. The positive predictive value was 100 percent and the negative predictive value 96.1 percent. The diagnosis of phyllodes tumor was missed by frozen section biopsy in three cases. The margins were informed in the 258 frozen section biopsies (79 percent) and in 59 cases (18 percent), these were positive for cancer. Conclusions: Frozen section biopsy is useful and reliable for cancer detection and margin status assessment in breast cancer surgery (RevMéd Chile 2009; 137: 1173-8).

Evolución clínica y radiológica de una mujer con histiocitosis pulmonar de células de Langerhans durante 18 años / Clinical and radiological evolution of a woman with pulmonary Langerhans cell histiocytosis during 18 years

The Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon pulmonary diffuse cystic disease. This review presents one case of PLCH diagnosed in a non-smoker female adolescent, who consulted for rapidly progressive dyspnea associated to left pleuritic pain that corresponded to a bilateral pneumothorax. The diagnosis was confirmed by surgical biopsy of the lung. Treatment with corticoids (prednisone) was indicated during the first year. After 18 years from diagnosis she presented an important clinical improvement, with a favourable but not complete radiological and spirometric improvement. The cases that make their debut with recurrent spontaneous pneumothorax are infrequent. The natural evolution of this disease is variable and the treatment is still controversial, been the immunosuppressive therapy, as corticoesteroids and cytotoxic agents of limited value, since are few studies that confirm their effectiveness.

La histiocitosis pulmonar de células de Langerhans (HPCL) es una enfermedad pulmonar difusa quística poco frecuente. En esta revisión, presentamos un caso de HPCL diagnosticada en una adolescente, no fumadora, que consultó por disnea rápidamente progresiva asociada a dolor pleurítico izquierdo, debido a un neumotorax bilateral. El diagnóstico se confirmó por biopsia quirúrgica. Se indicó tratamiento con corticoides (prednisona) durante el primer año. Tras 18 años de seguimiento destaca una importante mejoría clínica, con una evolución radiológica y espirométrica favorable, pero no completa. Los casos que se presentan con neumotorax espontáneo recurrente son raros. La evolución natural de esta enfermedad es variable y su tratamiento aún es controversial, siendo la terapia con inmunosupresores, tal como corticoesteroides y agentes citotóxicos de valor limitado, ya que son escasos los estudios que avalan su eficacia.