Ovarian steroid cell tumor, not otherwise specified: a case report and review of literature / 中华内分泌代谢杂志

We present a case of a postmenopausal women with hyperandrogenic symptoms and virilization signs , such as hirsutism, alopecia, acne and clitoromegaly, which was pathologically confirmed to be an ovarian steroid cell tumor, not otherwise specified(NOS). The levels of testosterone, dehydroepiandrosterone sulfate and estradiol in serum were increased, while the levels of luteinizing hormone and follicle stimulating hormone were decreased. Computed tomography(CT) scan and magnetic resonance imaging(MRI) identified a solid, left ovarian tumor and detected an additional tumor of hypodensity in the left adrenal gland. ACTH stimulation test, hCG stimulation test, adrenal and ovarian vein sampling indicated that excessive androgens were derived from the ovary. After the injection of gonadotropin hormone analogues(GnRHa), testosterone levels dropped to the normal range. Laparoscopic bilateral adnexectomy was performed, and pathology indicated NOS. The purpose of this report is to improve the understanding of NOS with hyperandrogenic presentation.

Frequency, distribution, and immunomorphologic characteristics of peripheral T-cell lymphoma, not otherwise specified in a tertiary care center in Southern India

Aims: In world literature, Peripheral T-cell lymphomas (PTCLs) constituted about 12% of non-Hodgkin's lymphomas (NHL) of which PTCL not otherwise specified (NOS) was the most common subtype. This study was undertaken to ascertain the frequency and to assess the morphologic and immunophenotypic characteristics of PTCL, NOS over a period of 5 years in a tertiary care referral center in Southern India. Materials and Methods: Slides and blocks of all PTCL, NOS were retrieved, and a detailed morphologic and immunophenotypic study using a wide panel of antibodies was done. Results: During this study, NHL constitutes 77.61% of all lymphomas. PTCL formed about 12.55% (251 cases) of all NHL. PTCL NOS was the most common subtype (30.68%). The most common site of involvement was lymph nodes (75%) followed by extranodal sites such as soft tissue (8.33%), gastrointestinal tract including oral cavity (6.67%), nasal cavity (5%), central nervous system (1.67%), lung (1.67%), and spleen (1.67%). PTCL, NOS showed a broad morphologic spectrum and had varied morphologic patterns with some mimicking reactive hyperplasia and some mimicking known type of T-cell lymphomas, B-cell lymphomas, and Hodgkin's lymphoma. Conclusions: PTCL, NOS constituted about 30.68% of all PTCLs in our institution during a 5-year period and was the second most common type of PTCL. Immunophenotyping using a wide panel of T-cell antibodies is necessary to distinguish PTCL, NOS from other lymphomas which they mimic, as they are known to carry a worse prognosis.

Clinical analysis of Hyper-CVAD/MA regimen and CHOP/CHOP-like regimen in the treatment of 78 patients with peripheral T-cell lymphoma, not otherwise specified (PT-CL-NOS) / 中国肿瘤临床

Objective:To compare the therapeutic efficacy and safety of Hyper-CVAD/MA regimen and CHOP/CHOP-like regimen in the treatment of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). Methods:The 78 primary PTCL-NOS patients who were initially diagnosed and treated in Tianjin Medical University Cancer Institute and Hospital and Tianjin Union Medical Center from June 2004 to June 2012 were retrospectively analyzed. The patients were then divided into two groups:Hyper-CVAD/MA group (n=21) and CHOP/CHOP-like group (n=57). Curative efficacies and toxicities were analyzed by Chi-square test, and survival was estimated by Ka-plan-Meier method. Results: In the Hyper-CVAD/MA group, complete response (CR) was 42.9%, overall response rate (ORR) was 85.7%, median progression-free survival (PFS) was 20 months, and the three-year overall survival (OS) was 56.9%. In the CHOP/CHOP-like group, the CR, ORR, and three-year OS were 28.1%, 59.6%, and 49.6%, respectively, and the median PFS was 13 months. Compara-tive analysis showed that the ORR and three-year OS were statistically significant (P0.05). The incidence rates ofⅢ/Ⅳneutrocytopenia and thrombocytopenia in Hyper-CVAD/MA group (66.7%and 61.9%, respectively) were significantly higher than those of the CHOP/CHOP-like group (22.8%and 14.0%, respec-tively) (P<0.05). Conclusion:Hyper-CVAD/MA regimen can achieve satisfactory efficacy in parents with PTCL-NOS, and toxicity can be controlled with granulocyte colony stimulating factor (G-CSF).

Non-mycosis Peripheral T-Cell non-Hodgkin Lymphoma involving the Skin.

A 64 years old male presented with reddish lesions all over the body of 1 month duration, high grade fever with evening rise of temperature and chills. No lymphadenopathy or hepatosplenomegaly were noted. Multiple infiltrated erythematous and hyperpigmented patches and plaques were present on the face, trunk and extremities along with few oral erosions. Histopathology from skin showed features of mycosis fungoides (MF). A further workup with Immunohistochemistry was suggestive of peri pheral T-cell lymphoma, not otherwise specified diag nosis (PTCLNOS). We report a case of PTCLNOS in a man mimicking MF clinically and histopathologically.

Sequential diffuse large B-cell lymphoma after peripheral T-cell lymphoma not otherwise specified: a case report and review of literature / 白血病·淋巴瘤

Objective To investigate the diagnosis and treatment of sequential diffuse large B-cell lymphoma (DLBCL) after peripheral T-cell lymphoma (PTCL).Methods A case with sequential DLBCL after PTCL was reported,and the characteristics and responses of this case were analyzed.The previous literature was reviewed in order to explain the mechanism and prognosis of such type of disease.Results This patient was diagnosed as PTCL not otherwise specified (PTCL-NOS) definitely,but after a period of treatment,DLBCL was developed as a second tumor.The characteristics and onset interval were just similar to those described in the literature,in which the mechanisms were mentioned as common effects of tumor cell,microenviroment and therapies.This patient got effects through the initial treatment,but considering the poor outcome by former researchers,the prognosis needed to be closely followed up.Conclusion Sequential development of EBV-unrelated DLBCL after PTCL-NOS is very rare,and the mechanism,therapy and prognosis need further investigation.

Prevalence of abnormal liver function tests and comorbid psychiatric disorders among patients with anorexia nervosa and eating disorders not otherwise specified in the anorexia nervosa DSM-IV criteria

<p><b>INTRODUCTION</b>Anorexia nervosa (AN) and eating disorders not otherwise specified (EDNOS) are on the rise in Singapore. Abnormal liver function tests have been reported for up to 12.2% of patients with AN. These patients are also known to present with comorbid psychiatric disorders. This study aims to investigate the correlation between body mass index (BMI) and the severity of abnormal liver function tests, and between BMI and the presence of comorbid psychiatric disorders.</p><p><b>METHODS</b>A retrospective cohort analysis of 373 patients diagnosed with AN or EDNOS at a tertiary hospital was performed. The clinical course of transaminitis and comorbid psychiatric disorders was correlated with the patient's BMI.</p><p><b>RESULTS</b>Patients with a BMI of ≥ 16.6 kg/m(2) at their first consult had a significantly lower risk of having comorbid psychiatric disorders (χ(2) = 32.08, p < 0.001). These patients were five times less likely to have comorbid psychiatric disorders as compared to patients from the other BMI groups (odds ratio [OR] 0.21). On the other hand, patients with a BMI of < 14.6 kg/m(2) had a significantly higher risk of having transaminitis (χ(2) = 72.5, p < 0.001). They were 11.1 times more likely to develop transaminitis as compared to patients with a BMI of ≥ 14.6 kg/m(2) (OR 11.05).</p><p><b>CONCLUSION</b>Severity of BMI can be used by clinicians as an indicator to assess for secondary psychiatric comorbidities and/or transaminitis during the first consultation. This could help reduce the morbidity and mortality rates in patients with AN or EDNOS.</p>

Juvenile Spondyloarthritis with Microscopic Colitis.

We report an adolescent girl with long standing spondyloarthritis and chronic diarrhea. Colonoscopy and biopsy revealed microscopic colitis. All serology and HLA-B27 were negative. This case is reported for its rarity and the need to evaluate gut in chronic arthritis to achieve clinical remission.

A Case of Primary Eyelid Peripheral T-Cell Lymphoma, Not Otherwise Specified

PURPOSE: To report a case of primary peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), of the eyelid. METHODS: A 48-year-old healthy male patient presented with a mass on the upper lid of 2 months in duration. The lesion was reddish, well-demarcated, oval shaped, and measured approximately 8 x 4 mm. The mass did not respond to incisional drainage and intra-lesional triamcinolone. An excisional biopsy for diagnosis was performed. RESULTS: On microscopic examination, a localized dense lymphocytic infiltration was observed in the subepithelial area, and cytologic atypia was observed under high power. On immunohistochemical examination, tumor cells were positive for CD3 but negative for CD20, CD30, CD56, k-light chain immunoglobulin, lambda-light chain immunoglobulin, and increased Ki-67 activity was noted. A histopathological diagnosis of PTCL-NOS was made. CONCLUSIONS: PTCL-NOS, which rarely occurs on the eyelids, commonly accompanies generalized lymphadenopathy and "B symptoms" such as fever and weight loss. Herein, the authors report a case of PTCL-NOS of the eyelid presenting as a rapidly growing solid mass in an otherwise healthy patient.

Effectiveness of Lamotrigine Adjunctive Treatment of Depressive Symptoms in Patients with Bipolar Disorder Not Otherwise Specified: A 52-Week Prospective Naturalistic Study / 대한정신약물학회지

OBJECTIVE: The pharmacotherapy of bipolar disorder not otherwise specified (BP-NOS) has been insufficiently studied. The aim of this prospective naturalistic study was to explore the effectiveness of lamotrigine adjunctive treatment in patients with BP-NOS. METHODS: Data from 50 patients diagnosed with BP-NOS were analyzed. On the basis of the prospective mood chart methodology, the efficacy of lamotrigine adjunctive treatment was assessed by changes in the mean Clinical Global Impressions-Bipolar Version (CGI-BP) depression scores. A paired t-test was used to test the statistical significance of the changes in CGI-BP depression scores. Repeated-measures analysis of variance (RM ANOVA) with simple effect analysis was performed to explore the sequential changes during a 52-week period. Cohen's d was calculated to measure the magnitude of the treatment effects on the changes in depression severity. Time to lamotrigine discontinuation was also calculated using the Kaplan-Meier estimates. Lamotrigine-associated adverse events were monitored every two weeks. RESULTS: A significant decrease, with a large effect size (Cohen's d=1.6), in the mean CGI-BP depression scores was associated with lamotrigine adjunctive treatment in intent-to-treat analysis (t=8.7, df=49, p<0.001). Twenty-four patients (48.0%) completed 52-week lamotrigine adjunctive treatment. Analysis of the data obtained from those completing the treatment revealed a large effect (Cohen's d=4.0) on improvement in the severity of depression (t=16.8, df=32, p<0.001). Sixty percent of patients achieved remission (n=30), and 64% of patients (n=32) showed some clinical response to lamotrigine adjunctive treatment. The mean time to lamotrigine discontinuation was 31.3+/-3.1 weeks (CI=25.2-37.4). Lamotrigine adjunctive treatment was well tolerated, with no serious rashes reported. CONCLUSION: Lamotrigine seems to be effective in the management of depressive symptoms in BP-NOS. Long-term use of lamotrigine was generally safe and well tolerated. Large-scale controlled trials might be needed to confirm the findings of this naturalistic study.

Dificultades en la evaluación y diagnóstico del autismo: Una discusión / Difficulties in assessing and measuring autism: A discussion

The detection of autism is very important because the lack of recognition of this disorder has elevated costs for the families, health care and education providers. Diagnosis is made frequently four or five years after parents notice the first signs. The reasons for this delay are many, but a common one is the lack of recognition of key symptoms that can lead to a more complex diagnosis assessment. Another reason is that screening and diagnostic instruments are not well known by primary caregivers in health and education systems, as these professionals are the first to hear parents' concerns. Moreover the instruments are not well known because the cost of acquiring them and receiving formal training is very high. The need to make comparable assumptions of this complex disorder makes it important to use the same instruments as other countries. Growing efforts for an early recognition have been made in recent years because early intervention programs benefit children with autism. In the last decade, important advances in the design of diagnostic and screening instruments have been made. These tools have primarily been used for clinical, epidemiological or research uses. In some countries their use has become routine in schools, leading to better detection and increasing prevalence rates of autism. Misdiagnosis is not uncommon in autism. Almost 60% of children with Asperger disorder first receive an erroneous diagnosis of attention deficit disorder, oppositionistic or bipolar disorder. Autism presents with a bizarre clinical picture during the years in which many thought it was untestable. Gradual characterization of behaviors and studying different aspects of the symptomatology had led to a better comprehension and descriptions. Most authors have incorporated this knowledge to design reliable instruments. The most common behaviors explored are: protodeclarative pointing, joint attention, repetitive/ stereotyped movements and absence of characteristic symbolic play. This target behavior can be explored through the diverse rating scales and interviews. The instruments are very diverse and varing form. There are rating scales for parents to record their children symptoms and observation schedules to be completed by a clinician or trained professional for that purpose. The best approach is to combine modalities to include as much information as possible. CHAT (Checklist for Autism in Toddlers) is a brief screening instrument intended to detect autism in toddlers. The first part consists of nine questions for parents to complete, while the second part is an observation schedule with five brief age-appropriate interactions with the children. This instrument is an important antecedent of more sophisticated and expanded play observation schedules. Checklist for Autism in Toddlers Modified (CHAT M) is a modified version which consists of an expansion of the parent questionnaire by eliminating the observational section. The Childhood Autism Rating Scale (CARS) is another instrument which assesses the severity of autism. This instrument is rated by clinicians or by trained observers. CARS was designed before DSM IV criteria were published so it does not contain an algorithm to distinguish between different developmental problems. In spite of this limitation, it is the most used rating scale for autism diagnosis. The Child Behavior Checklist (CBCL/1.5-5) is a broad band rating scale which evaluates psychopathology of children between 18 months and five years old. It has a DSM oriented subscale to evaluate developmental problems such as autism or Asperger disorder. It also contains a withdrawn subscale which has proven to be useful as demonstrated by some studies done with the CBCL/4-18. This instrument also allows assessing other associated problems common in autistic children such as attention problems, depression and anxiety. The Language Developmental Survey (LDS) associated to this rating scale, gives the opportunity to screen vocabulary for the identification of language delays, which are common in children with pervasive developmental disorders. It was necessary to have more structured instruments to diagnose autism and not only for screening purposes, so in 1989 the first diagnostic interview was published. The instrument has gone through an extensive review and creative process which has led to the most important tools for diagnosing autism in adults and children. The Autism Diagnostic Interview (ADI) was published in 1989 and correlated to the ICD-10 definition of autism. The original ADI was intended primarily for research purposes, providing behavioral assessment for subjects with a chronological age of at least five years and a mental age of at least two years. The ADI explores three key domains defining autism: (1) reciprocal social interaction, (2) communication and language, and (3) repetitive, stereotyped behaviors. The Autism Diagnostic Interview Revised ADI-R is a semi/ standardizer interview shorter than the ADI, which has been developed for clinical use. It is more appropriate for younger children than the ADI. The ADI-R takes from 2 to 3 hours to administer and can be used with children as young as two years of age (with a mental age greater than 18 months). It explores information about the child functioning in the present and the past. It contains an algorithm based on DSM criteria for autistic disorder, and allows for distinguishing between autistic disorder and non autistic disorder. Pre Linguistic Autism Diagnostic Observation Schedule (ADOS-PL) is a modified version of the ADOS used to diagnose young children (under the age of six years) who are not yet using phrase speech. It is a semi-structured assessment of play, interaction, and social communication and takes about 30 minutes for a trained clinician to administer. The Autism Diagnostic Observation Schedule-Generic (ADOS-G) is a standardized play observation schedule. Through structured play materials and activities promoted by the examiner, social interactions are rated for common autistic features like joint attention, protodeclarative pointing, quality of reciprocal social interaction and symbolic play. Different modules are available from one to four, with specified criteria to match the participants' developmental and language level. It contains an algorithm related to the DSM IV domains of an Autistic Disorder or PDD-NOS. The ADI, ADI/R, ADOS PL, and ADOS G are considered the gold standards for autism diagnosis. There are important reliable instruments for diagnosing autism but extensive training is needed to obtain useful diagnostic information. Since these instruments are very recent, they have not been validated in some countries and neither their cultural bias has been investigated. It is not enough to assess autistic symptoms only for diagnostic purposes; patients need further evaluation to determine their psychosocial functioning, cognitive abilities, and language delay or deviations. The information from these assessments is very important for planning well designed interventions. Even though there is a growing interest in perfecting these modern instruments, diagnosis cannot rely exclusively on them. They are important tools to facilitate the diagnosis, but broader assessment should be pursued. It is important to validate and culturally adapt these instruments so different countries can utilize the same tools and research results can be comparable. In the future more rating scales, observation schedules and diagnostic interviews will be developed for assessing Asperger disorder, to be used in genetic studies, for assessing broad band syndromes. Better cognitive measures will be necessary to evaluate psychosocial impact. But this growing specialization will increase costs so it is important to develop briefer and more cost-effective methods to evaluate persons with autism. The availability of these tools will guarantee early diagnosis and treatment not only for research purposes but for identification in the community.

La detección del autismo en México es muy importante ya que la falta de reconocimiento de este trastorno tiene costos muy elevados para las familias y los prestadores de servicios de salud y educación. Muy a menudo el diagnóstico de autismo se realiza cuatro o cinco años después de que los padres observan los primeros síntomas. Las razones para este reconocimiento tardío son diversas; pero una de las principales es la falta de identificación de síntomas clave que obliguen a una evaluación diagnóstica en forma. Otro motivo es que en nuestro país son poco conocidos los instrumentos de tamizaje y diagnóstico por parte de los profesionistas primarios como maestros y médicos familiares, quienes son los primeros en escuchar las quejas y preocupaciones de los padres. Aun en contextos más especializados, estas herramientas son poco conocidas pues su adquisición y aplicación es un proceso complejo y costoso que a menudo debe realizar el profesionista por su cuenta. A pesar de estos inconvenientes, en años recientes se han realizado grandes esfuerzos para el reconocimiento del autismo puesto que hay evidencias de que las intervenciones tempranas mejoran el pronóstico en estos niños. En la última década se han realizado avances muy importantes en el diseño de instrumentos de diagnóstico y tamizaje, a los que se han utilizado con propósitos de investigación clínica y epidemiológica. En algunos países su uso se ha vuelto una rutina en las escuelas y se ha logrado una mayor detección de autismo por lo que se han elevado las tasas de prevalencia. Los instrumentos son muy diversos, pueden ser listas de autoinforme dirigidos a los padres para que registren los síntomas de los niños, o cédulas de observación para ser completadas por el clínico o el personal entrenado para tal propósito. Lo mejor es el uso mixto de instrumentos para obtener la mayor cantidad de información posible como es el caso del CHAT que incluye una sección de interrogatorio y otra sección de observación con actividades que el niño debe desarrollar. Este instrumento es precursor de actividades sencillas y creativas con un componente lúdico, diseñadas con el propósito de evaluar al niño preescolar. Hoy este es un importante antecedente de otros instrumentos más elaborados. El cuestionario para el autismo en niños preescolares modificado CHAT M es una versión modificada del el cuestionario para el autismo en niños preescolares (CHAT) que consiste en una expansión de la sección de interrogatorio para el padre, con un formato de autoinforme que parte de la eliminación de la sección de observación. Otros instrumentos miden la gravedad del autismo como la Escala de Evaluación de Autismo Infantil (CARS), dirigida al clínico que evalúa la intensidad del autismo. La lista de síntomas del niño de 1.5-5 (CBCL/1.5-5) es un instrumento de banda ancha que evalúa la psicopatología general en niños con edad entre 18 meses y cinco años; contiene una subescala de problemas del desarrollo que sirve como tamizaje para evaluar el autismo y el trastorno por Asperger con base en los criterios del DSM. Se han diseñado y se han perfeccionando paulatinamente varias entrevistas de diagnóstico. La entrevista de diagnóstico de autismo (ADI), la entrevista de diagnostico para el autismo revisada (ADI-R), la cédula prelingüística genérica de observación para el autismo (ADOS PL), y la cédula de observación genérica para el autismo (ADOS G) son escalas consideradas standard de oro para el diagnóstico del autismo. Conforme se han mejorado las propiedades psicométricas, de los instrumentos, éstos también se han ajustado para cubrir las necesidades de evaluación de los pacientes autistas con un amplio rango de edad, destreza verbal y cognitiva. Como resultado, podemos contar con instrumentos confiables y adecuados para una población con necesidades muy diversas; estas herramientas nos han demostrado que un constructo tan complejo y amplio como el autismo se puede medir. En este artículo se presenta una breve revisión de la evolución histórica de la clasificación acorde a los criterios del DSM y a la descripción de los principales instrumentos de diagnóstico, y los datos de su validez y confiabilidad.

A Case of Steroid Cell Tumor, Not Otherwise Specified, with Massive Ascites / 대한산부인과학회잡지

Steroid cell tumors, not otherwise specified, are rare ovarian sex cord-stromal tumors with malignant potential. The majority of these tumors produce steroids (testosterone is the most common) and various virilizing symptoms such as hirsutism, temporal balding and amenorrhea, may appear in patients. Executive history taking, physical examinations, CT or sonography and hormonal studies are helpful in the diagnosis, but the confirmation of diagnosis is made via a staging operation and pathology. Treatments include operation, chemotherapy (i.e., BEP), GnRH agonist therapy and radiotherapy. We experienced a case of steroid cell tumor, not otherwise specified, with massive ascites, and elevated CA125, which we wish to report with a brief review of the literature.