Exploring the unusual: a testosterone-secreting ovarian tumor

ABSTRACT Ovarian steroid cell tumors are rare, representing less than 0.1% of all ovarian neoplasms. Among the myriad causes of hirsutism, ovarian tumors account for 1% of the reported cases. We present the case of a 49-year-old parous postmenopausal woman who sought medical attention for hirsutism for 2 years. This case illustrates the unusual and interesting connection between rare ovarian pathology and the clinical manifestation of hirsutism in a postmenopausal patient. Her ultrasonography and MRI showed a right adnexal mass of solid-cystic consistency with thin septations. Her laboratory workup revealed high levels of total testosterone of 256 ng/ml (8.4-48.1ng/ml) and free testosterone of 7.36 pg/ml (0.2-4.1 pg/ml), while DHEAS - 234 µg/dl (35.4-256 µg/dl) and CA125 - 15.8U/L (0.0-35 U/L) were in the normal range. She underwent exploratory laparotomy with a total abdominal hysterectomy and oophorectomy. Histopathological examination and immunohistochemistry conclusively established the presence of a steroid cell tumor, specifically classified as "Not Otherwise Specified"(NOS), in the right ovary.

An Observational Study on Cutaneous Appendageal Tumours with Pilar Differentiation

Background: Cutaneous appendageal tumours belong to a diverse group of tumours with specific histo pathology.The aim of this study is to determine the pattern, age,gender and site distribution of Pilar differentiation tumours. Material & Methods: The study was conducted in the department of Pathology,Government Medical College Srinagar for a period of 18 months. It was an observational cross sectional study.Formalin fixed,paraffin embedded tissue sections were stained with hematoxylin and eosin stain for histopathological analysis. Results: A total of 112 cases of Pilar tumours were studied.108 were benign and 4 were malignant with male to female ratio of 1.07:1. The maximum number of benign cases were observed in 11 -20 years of age group and the malignant tumours age ranges from 35-45 years and the tumour usually presented in the eighth decade.Head and Neck was the most common site. Conclusion: Histopathological examination of Pilar Tumours is the gold standard to differentiate between benign and malignant tumours. It is also useful for exact categorization of cutaneous appendageal tumours.

Analgesic effect and safety of using an epidural analgesia pump versus an intravenous analgesia pump for uterine artery embolization in the treatment of uterine fibroids / 中国基层医药

Objective:To investigate the analgesic effect and safety of using an epidural analgesia pump versus an intravenous analgesia pump for uterine artery embolization in the treatment of uterine fibroids. Methods:Fifty patients with uterine fibroids undergoing uterine artery embolization admitted to The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University from January 2019 to December 2021 were included in this study. They were divided into an observation group and a control group ( n = 25/group). Patients in the observation group used an epidural analgesia pump for pain relief, while patients in the control group used an intravenous analgesia pump for pain relief. At 1, 6, 12, and 24 hours after surgery, pain severity was compared between the two groups using the Visual Analogue Scale. Comfort level was compared between the two groups using the Bruggemann Comfort scale. Before and after surgery, respiratory rate, heart rate, blood pressure, and adverse reactions were compared between the two groups. Results:At 1 hour after surgery, the Visual Analogue Scale score in the observation group was significantly lower than that in the control group [3.00 (2.00, 5.50) vs. 7.00 (6.00, 8.00), Z = -3.84, P < 0.05]. At 6, 12, and 24 hours after surgery, there was a significant difference in the Visual Analogue Scale score between the two groups (all P > 0.05). Within 24 hours after surgery, the use of opioid analgesics in the observation group was less than that in the control group [16.00% (4/25) vs. 88.00% (22/25), χ2 = 25.96, P < 0.001]. At 1 hour after surgery, the Bruggemann Comfort Scale score in the observation group was significantly higher than that in the control group [0.00 (0.00, 0.50) vs. 0.00 (0.00, 0.00), Z = 2.08, P < 0.05]. At 6, 12, and 24 hours after surgery, there was no significant difference in the Bruggemann Comfort Scale score between the two groups (all P > 0.05). After surgery, heart rate was significantly decreased in each group compared with before surgery (both P < 0.05). There were no significant differences in respiratory rate and mean arterial pressure between the two groups before and after surgery (both P > 0.05). There were no significant differences in the incidences of postoperative nausea, vomiting, and fever between the two groups (all P > 0.05). Conclusion:The epidural analgesia pump used for uterine artery embolization in the treatment of uterine fibroids has a better analgesic effect and provides more comfort and is safer than the intravenous analgesia pump. The former is worthy of clinical promotion.

Analysis on the implementation effect of single disease payment policy for day surgery based on difference-in-differences model / 中华医院管理杂志

Objective:To analyze the implementation effect of single disease payment policy for day surgery (hereinafter referred to as the policy), for references for the reform of medical insurance payment.Methods:By collecting the information of inpatients from 2017 to 2019 in a tertiary hospital, the research group took patients with colorectal benign tumor and nodular goitre as the policy implementation group and the control group respectively. 2017-2018 was the pre implementation stage of the policy, and 2019 was the post implementation stage of the policy. The difference-in-differences (DID) model was used to analyze the changes in indicators such as length of stay and hospitalization expenses after policy implementation, under whether the policy is implemented or not, as well as before or after policy implementation.Results:A total of 2 419 patients were included, including 927 patients with nodular goiter in the control group and 1 492 patients with colorectal benign tumors in the policy implementation group (688 patients before the policy implementation and 804 patients after the policy implementation). The results of DID showed that the hospital days for patients with colorectal benign tumor decreased by 56.53%, the hospitalization expenses decreased by 26.51%, the out-of-pocket expenses decreased by 26.66%, the treatment expenses increased by 11.96%, the drug expenses decreased by 50.29% and the consumables expenses decreased by 20.23% after the implementation of the policy.Conclusions:The implementation of the policy could reduce length of stay, hospitalization expenses and out-of-pocket expenses, optimize the structure of hospitalization expenses, improve the efficiency of hospital diagnosis and treatment, and help the hospital realize its transformation from a size expansion to a quality and benefit expansion.

STUDY OF CLINICAL PROFILE AND MANAGEMENT OF INTRADURAL EXTRAMEDULLARY SPINAL CORD (IDEM) TUMOURS (CONDUCTED AT A TERTIARY CARE CENTER IN CENTRAL MAHARASHTRA)

Aims and objectives: This study aims to analyse the complete profile of patients diagnosed and treated as IDEM spinal cord tumours. The factors studied include age, sex, clinical presentations, histopathology, surgical outcome and post operative recurrence. It is a single center prospective study conducted over a peri Materials and method: od of 18 months. Patient demographics, severity and duration of symptoms, and tumor characteristics (anatomical and pathological) in all operated spinal IDEM tumors were collected. The neurological findings obtained during the preoperative stage and the postoperative follow-up were evaluated according to the Frankel and Nurick grading. The back pain was assessed with help of the Denis pain scale (DPS). Data collected throughout preoperative and postoperative examination and investigations were analyzed and submitted to statistical procedures. Our study Results and analysis: included30 patients, majority being young adults between 20 to 30 years (46.66%). The male and female ratio in our study was 2:1. Predominant presentation was sensory symptoms like numbness, paraesthesia in extremeties (66.66%), followed by pain in back, limbs (33.33%). The most common location found was dorsal cord (50%) followed by lumbar(36.66%). The histopathological examination showed meningioma to be the most common (56.66%), followed by schwannoma (20%), and neurofibroma (16.66%). Pseudomeningocoele was the only complication encountered. Out of 30 patients we had operated, we could achieve total excision in 27 patients (90%). There was no postoperative worsening of symptoms, and no recurrence. The clinical and pre and postoperative profile suggested Summary and Conclusion: IDEM spinal cord tumours were common in 3rd decade, with male predominance. Commonest location was dorsal area with meningioma being the commonest histopathological finding.

Histopathological pattern of Orbito-Ocular Lesions, a retrospective hospital-based study spanning 15 years

Background: Many previous studies on orbito-ocular lesions are skewed in favour of the neoplastic lesions in general and the malignant lesions in particular. This, therefore, creates a vacuum on the spectrum of these lesions, thus may result in problematic diagnostic bias by the ophthalmologist and pathologist. Objective: To give the spectrum and relative frequencies of orbito-ocular biopsies and by extension orbito-ocular lesions/diseases at the University of Benin Teaching Hospital (UBTH). Materials and Methods: A retrospective descriptive study of all cases of orbito-ocular biopsies with histopathologic diagnosis. Results: There were 236 orbito-ocular biopsies. The male to female ratio was slightly in favour of the females. Orbito-ocular biopsies had a wide age range that spanned from the 1st to 10th decade, mean age in the 3rd decade (20-29years) and a peak age in the 1st decade (0-9 years). The neoplastic lesions were the prevalent indication for orbito-ocular biopsies (63.72%) while the conjunctiva (58.10%) was the most common site for orbito-ocular biopsies. Conclusion: This study noted a wide array of orbito-ocular lesions for which biopsies were done for histopathological diagnosis. This we hope will in no small measure increase the diagnostic precision of the ophthalmologist and the pathologists in our own environment

The effect of CAM comprehensive nursing interventions on the improvement of quality of life to patients with breast cancer and gynaecological tumours

Breastcancerin females, which is consideredthe most dreadfuldisease in India andthe worldas compared toother gynaecological cancers,demands extensive care and proper medicationin order to control itsprogressive growth. In addition to the conventional care ofthe patients, Complementary andAlternative Medicine(CAM)is administeredin a controlled way through proper guidance and counselling in orderto attainimprovedphysical andmental health forthe patients.Objective: The aim of the study wasto assess the effectiveness of CAMcomprehensive nursing interventionsand their benefit forpatients who havebreast cancer and gynaecological tumours.Methods: Statistical data was used to map the adult womendiagnosed with breast and gynaecological cancer and who were set to start new chemotherapy treatments. A total of 450 patients from different states of East India were enrolled in the studyover a period of four years. The patients wereselectedbased on their preference for undergoing CAM.The research was conducted usingacross-sectionalanonymous self-administered questionnaireto examine women's perspectivestowards the use of CAM and itseffect on their mental andphysical health.Results:42%of the women preferred theuseofCAM astheir alternative treatment. Breast cancer patients disclosed that 48.1% of them had used CAM and 39% of women with gynaecological cancersstated that they had usedCAM. The results further indicated a less frequent deteriorationin the health of CAM users(38.4%) thantonon-users(55%). In terms of those who utilizedvitamins and nutritional diets,60% of the participants reported using a proper diet, including antioxidants, minerals, vitamins and herbs etc. 37% opted for spiritual healing through yoga, 26% utilized energy healing, 42.4% utilized acupuncture,72% preferred massagesand 23% of patients utilized chiropractic methods.The use of CAM was foundmainlyin the patients with proper education and awareness and those with a family history of cancer who were not receiving the necessary care from their previous primary physician.Conclusion:CAM still requiresextensive research in terms of its applications in dealing with patients and in orderto successfully launch programmes aimed at promoting its useworldwide andto eradicate all the other false notions about it.[Ethiop. J. Health Dev. 2022; 36(2):000-000]Keywords: Breast Cancerand gynaecological tumours,Complementary And Alternative Medicine (CAM), Complementary Oncology,Quality Of Life, Nutritional Diet and Spiritual Healing, HRQL (Health-Related Quality Of Life).IntroductionCancer has been the primaryconcern in Indiafrom the onset,resulting in thousands of deaths due to the lack of adequatemedication and therapy.Breast cancer is considered to bea common invasive form of cancer which is responsible for the second highest mortality rate among the primaryfatal conditions of cancer-causing deaths in women. Under the National Cancer Registry Program,the breast, cervix, uteri,and oral cavity(7)are the most common cancer sitesin women. According to statistics, 50-60% of all cancers amongIndian women are commonly found inthe cervix uteri, breast, corpus uteri and ovaries,which are the main organs forcancer invasion. The percentage of women who experience these types of cancer is increases, with more women becoming susceptible to breast cancer, in women up to 3-8% suffer fromovarian cancer,0.5-4.8%have cancer of the corpus uteri, 1-3% have vulva and/or gestational trophoblastic tumours and a staggering75,000 or more women have breast cancer. With the advancements of the disease through time,research programs were also improved in order to enable the improvement of existing measures and/or the development of new measures aimed at combating the diseas and decreasing mortality rates. Apart from the conventional chemotherapy technique,various other programs have been introduced andencorporated into treatment regimes in order to improve the lives of those who are affected by cancer.Complementary andAlternative Medicine (CAM) emphasizesthe methods and practices that are therapeutic and which help diagnose or curethe disease,these methods are intended tocomplement the conventional methods and can be used in place of mainstream medicine. Women with breast cancer and other gynaecological tumoursare increasingly inclined towards the use of CAM. With the increasing number of cancer reports in Indiaand the world,and with the principal amount leading to mortality, women with proper education and awareness are inclined to choosealternative methods rather than the old conventional ones. The side-effects resulting from the chemotherapy are immense and toxic,which is one of the reasons women are inclined to choose.Various research projects and cumulative studies have been conducted in order to review and discoverthe

Detection of Neuroendocrine Tumour of Sphenoid Sinus on the Gallium-68 DOTATATE PET/CT: A Rare Entity

@#Neuroendocrine tumours (NETs) are a category of neoplasm that is characterised by its phenotypic and heterogeneity. The occurrence of this type of neoplasm in the nasal cavity and paranasal sinuses is extremely rare accounting for only 0.2-0.8% of all cancers. NET tends to expresses somatostatin receptors (SSTR) and owning to this unique characteristic, molecular imaging has been able to detect these tumours using radiolabelled somatostatin analogue agent. Gallium-68 (Ga-68) DOTATATE PET/CT is an example of SSTR imaging and has been shown to be of importance in the assessment and staging of NET. We present a case of a rare sphenoid sinus NET in a 45-year-old gentleman whom initially presented with persistent left eye pain which led to visual loss. We described the utilization of Ga-68 DOTATATE PET/CT in the diagnosis and staging of this patient which in turn dictated treatment approach.

Molecular genetic analysis of primary renal epithelial tumours with granular oncocytic cytoplasms / Análisis genético molecular de tumores epiteliales renales primarios con citoplasma oncocítico granular

SUMMARY: The group of primary renal tumours with granular-oncocytic cytoplasm is a very heterogeneous group, in its histological origin and biological behavior resulting in many diagnostic problems. In this study 57 renal epithelial tumours with granular oncocytic cells were analyzed using fluorescence in situ hybridisation (FISH), array comparative genomic hybridisation (aCGH) and polymerase chain reaction (PCR). The results of analysis in renal oncocytoma (RO) did not indicate the presence of the gene mutations or chromosomal abnormalities. Sporadic renal hybrid oncocytic/chromophobe tumours (HOCT) had multiple numerical aberrations of chromosomes 1, 2, 6, 9, 10, 13, 17, 20, 21 and 22. This type of tumour had no mutations in the VHL, c-kit, PDGFRA, and FLCN genes. Oncocytic papillary renal cell carcinoma (O-PRCC) had numerical abnormalities of chromosomes 7 and 17 and the loss of the Y chromosome. Cytogenetic analysis of 20 pigmented microcystic chromophobe renal cell carcinomas (PMChRCC) showed monosomy as the most frequent aberration in all analyzed chromosomes 1, 2, 5, 10, 13, 17 and 21. One case of chromophobe renal cell carcinoma (ChRCC) with hyaline globules had a mutation in the distal part of exon 3 of the VHL gene. Absence of genetic disorders in usual RO is common result, but we have established absence of genetic disorders even in rare variants. Variety of genetic alterations detected in sporadic renal HOCT proves it to be a separate entity, not a variant of ChRCC, while PMChRCC is an uncommon variant of ChRCC. O-PRCC is a subtype of papillary renal cell carcinoma.

RESUMEN: El grupo de tumores renales primarios con citoplasma granular-oncocítico es un grupo muy heterogéneo, en su origen histológico y comportamiento biológico, resultando en problemas de diagnóstico. En el estudio se analizaron 57 tumores epiteliales renales con citoplasma oncocítico granular mediante hibridación fluorescente in situ (FISH), hibridación genómica comparativa de matriz (aCGH) y reacción en cadena de la polimerasa (PCR). Los resultados del análisis en oncocitoma renal (RO) no indicaron la presencia de mutaciones genéticas ni anomalías cromosómicas. Los tumores oncocíticos / cromófobos híbridos renales esporádicos (HOCT) tenían múltiples aberraciones numéricas de los cromosomas 1, 2, 6, 9, 10, 13, 17, 20, 21 y 22. No se observaron mutaciones en este tipo de tumor en el VHL, c-kit, PDGFRA y genes FLCN. El carcinoma de células renales papilar oncocítico (O-PRCC) tenía anomalías numéricas de los cromosomas 7 y 17 y la pérdida del cromosoma Y. El análisis citogenético de 20 carcinomas de células renales cromófobos microquísticos pigmentados (PMChRCC) mostró que la monosomía era la aberración más frecuente en todos los cromosomas analizados 1, 2, 5, 10, 13, 17 y 21. Un caso de carcinoma de células renales cromófobo (CCRc) hialino tenía una mutación en la parte distal del exón 3 del gen VHL. La ausencia de trastornos genéticos en la OI habitual es un resultado común, pero hemos establecido la ausencia de trastornos genéticos incluso en variantes raras. Varias alteraciones genéticas detectadas en esporádica HOCT renal demuestran que es una entidad separada, no una variante de ChRCC, mientras que PMChRCC es una variante poco común de ChRCC. O-PRCC es un subtipo de carcinoma papilar de células renales.

Opinions on the reclassification of pulmonary adenocarcinoma in situ in the fifth edition of WHO classification of thoracic tumours / 中国胸心血管外科临床杂志

@#Pulmonary adenocarcinoma in situ is reclassified as precursor glandular lesions in the fifth edition of WHO classification of thoracic tumours, causing widespread attention and heated debate among domestic thoracic oncologists, radiologists, pathologists and surgeons. We would like to comment on the topic and make a few suggestions on the management of pulmonary nodule during lung cancer screening. We are open to all suggestion and welcome debates.

Enzyme-Linked Immunosorbent Assay Detection For Malignancy Using Anti-P53 Antibodies

@#Tumour protein 53 (p53) plays an important role in the instruction of the cell cycle. In a variety of transformed cell lines, tumour protein is expressed in high amounts, and it is believed to contribute to transformation and malignancy. This research aimed to detect the anti-p53 antibodies in sera of patients with various malignant tumours and to evaluate the sensitivity and specificity of an enzyme-linked immunosorbent assay (ELISA). A case-control study was conducted on samples from 49 patients with various types of malignant tumours at Sultanah Bahiyah Hospital, Alor Setar, Kedah, Malaysia, and 32 healthy control cases with non‐malignant disease collected from Universiti Sains Malaysia clinic, Penang, Malaysia. The antibodies against p53 protein in the serum samples were analysed using the commercial ELISA kit, Calbiochem® p53- ELISAPLUS. The results showed that the rate of anti-p53 antibodies in patients with various malignant tumours was 13 out of 49 (26.5 %), compared with only 2 out of 32 (6.25%) in healthy controls (p < 0.001). The sensitivity of this kit reached 28.6% and the specificity was 93.8%. In conclusion, these results suggest that the anti-p53 antibodies can be detected in different sera of malignant tumour patients and the ELISA kit is highly specific; nevertheless, its discrimination power is not perfect because of its low sensitivity to determine the anti-p53 antibodies.

Pediatric Adrenocortical Oncocytoma presenting as Cushing’s Syndrome and Peripheral Precocious Puberty:A case report and review of literature / Journal of the ASEAN Federation of Endocrine Societies

@#Oncocytic adrenocortical tumours (OATs) or oncocytomas are extremely rare and are usually benign and nonfunctional. We report the case of a 4-year-old male with a right-sided, functional oncocytic adrenocortical adenoma, who presented with precocious puberty and Cushing’s syndrome. After work-up, the patient underwent laparoscopic adrenalectomy. The excised adrenal mass weighed 21 g and measured 3.5 cm in maximum dimension. Histological examination demonstrated no features suggestive of aggressive biological behaviour. The patient had no features of recurrent or metastatic disease and had prepubertal testosterone levels with suppressed hypothalamic-pituitary-adrenal axis twelve months after the surgery. A discussion of this case and a review of the literature on functional OATs in the pediatric population are presented.

Management of Recurrence of Ameloblastoma and Odontogenic Keratocyst: A Cross-Sectional Study / Manejo de la recurrencia del ameloblastoma y keratoquiste odontogénico: Estudio de corte transversal

Abstract Objectives: Odontogenic keratocyst (OKC) and ameloblastoma are slowly growing and locally invasive tumors with high recurrence rate. The aim of this study was to investigate the clinicopathologic features of recurrent ameloblastoma and OKC cases, and evaluate outcomes of our treatments in terms of recurrence. Material and Methods: A total of 23 patients with confirmed recurrent ameloblastoma or OKC and treated in our clinic within eleven years period were reviewed retrospectively. Results: Eleven recurrent OKC cases and twelve recurrent ameloblastoma cases were included. Most recurrences occurred within five years after the initial treatment (69.6%). Enucleation had the highest recurrence rate among the first treatments (18/23). All recurrences were located in the mandible, with one exception (22/23). All recurrent OKCs were multilocular. Different histopathologic subtypes of ameloblastoma were seen in our study, follicular ameloblastoma was the most common (8/12). The mean diameter of the lesions was 4.3 cm (ranging from 2 cm to 7 cm). Statically significant relation was found between location and diameter of lesion and year of recurrence onset (p=0.004; p=0.026). We performed radical treatments in these cases (ten patients underwent marginal resections, and thirteen patients underwent segmental resection), and no recurrence was observed during the follow-up period. Conclusion: Previous inadequate surgical procedures were the most important cause of recurrence. Marginal or segmental resection with safety margins is the best method to treat recurrences of OKC or ameloblastoma cases.

Resumen Objetivo: Los keratoquistes odontogénicos (KQO) y ameloblastomas son tumores invasivos de lento crecimiento local con una alta tasa de recurrencia. El propósito de este estudio fue investigar las características clínico patológicas de los casos de ameloblastoma y KQO recurrentes y evaluar los resultados de tratamientos en término de recurrencia. Materiales y Métodos: Un total de 23 pacientes con casos confirmados de ameloblastomas o KQO recurrentes tratados en nuestra clínica en un período de once años fueron incluidos. Resultados: Once casos de KQO recurrentes y doce ameloblastomas recurrentes fueron incluidos. La mayoría de las recurrencias ocurrieron en los primeros 5 años posteriores al tratamiento inicial (69.6%). La enucleación mostró la tasa de recurrencia más alta entre los tratamientos iniciales (18/23). Todas las recurrencias se presentaron en la mandíbula excepto por un caso (22/23). Todos los KQO fueron multiloculares. Distintos subtipos histológicos del ameloblastoma fueron detectados en el estudio y el ameloblastoma folicular fue el más común (8/12). El diámetro promedio de las lesiones fue de 4.3cm en un rango de 2cm a 7cm. Una relación estadísticamente significativa se encontró entre la ubicación y el diámetro de la lesión y el tiempo de aparición de la recurrencia (p=0.004; p=0.026). Se realizaron tratamientos radicales en los siguientes casos, diez pacientes tuvieron resecciones marginales y trece pacientes resección segmental; no se observaron recurrencias en el período de seguimiento. Conclusión: Procedimientos previos inadecuados fueron la causa más relevante de recurrencia. Resección marginal o segmental con márgenes de seguridad son el mejor método para tratar casos de ameloblastoma y KQO recurrentes.

Rabdomioma intracardiaco: presentación clínica y opciones terapéuticas actuales a propósito de tres casos / Cardiac rhabdomyoma: Clinical presentation of three cases and current therapeutic options

Resumen Hasta hace algunas décadas los tumores cardiacos tenían solo un interés académico dada su baja tasa de presentación; sin embargo, con el advenimiento del baipás cardiopulmonar, el manejo quirúrgico comienza a ser posible y, más recientemente, junto con la investigación farmacológica, contribuyen al desarrollo de nuevas estrategias de tratamiento para estos pacientes. La incidencia estimada de los tumores cardiacos es baja y tiene un comportamiento generalmente benigno y manifestaciones clínicas inespecíficas. Estos tumores en la edad pediátrica se asocian a esclerosis tuberosa, una enfermedad de patrón de herencia autosómica dominante que se caracteriza por una alteración en la diferenciación y multiplicación celular (hamartomas) en diferentes sistemas. Desde el punto de vista cardiaco, el grado de morbilidad está condicionado por el tamaño y la localización tumoral dentro de la cavidad, y tiene riesgo de obstruir los tractos de salida ventriculares, alterar la función valvular o producir trastornos de conducción. Algunos pacientes solo requieren seguimiento clínico durante el transcurso de su vida, pero existe la posibilidad de regresión tumoral; otros se benefician de una resección quirúrgica por su impacto hemodinámico o alteraciones del ritmo cardiaco. Sin embargo, no todos son candidatos quirúrgicos, en cuyo caso el manejo con inhibidores m-TOR ha surgido como una alternativa terapéutica. Se presenta el caso de tres neonatos con esclerosis tuberosa y rabdomioma cardiaco, diagnosticados en etapa prenatal, a quienes se les realizó un abordaje terapéutico individualizado, basado en las opciones actuales disponibles para este grupo de pacientes.

Abstract Up to a few decades ago, cardiac tumours were only of academic interest given their low presentation rate. However, with the advances in cardiopulmonary, surgical management began to be possible, and more recently, they contribute to the development of new treatment strategies for these patients. The estimated incidence of cardiac tumours is low and are generally benign with non-specific clinical signs and symptoms. In paediatrics, these tumours are associated with tuberous sclerosis, a disease with a dominant autosomal inheritance pattern, which is characterised by an alteration in cell differentiation and multiplication (hamartomas) in different systems. From a cardiac point of view, the morbidity level is determined by the size and location of the tumour within the cavity, the risk of obstructing ventricular outflow tracts, alterations in valvular function, and conduction disorders. Some patients only require clinical follow-up all their lives, as there is the risk of tumour regression. Others may benefit from surgical resection due to its haemodynamic impact or changes in cardiac rhythm. However, not everyone is a candidate for surgery, in which case, management with m-TOR inhibitors has emerged as a therapeutic alternative. The case is presented on three neonates with tuberous sclerosis and a cardiac rhabdomyoma, diagnosed in the prenatal stage. An individualised therapeutic approach was made based on the current options available for this group of patients.

Primary retroperitoneal mucinous cystadenocarcinoma: a rare surgico-pathological entity

Ovarian cancer (OC) is the seventh most common cancer for females in the world. Epithelial OC is the most predominant pathologic subtype (85%-90%), with five major histotypes- serous, mucinous, endometrioid, brenner and clear cell tumour. Each have three pathological subtypes ie benign, borderline and malignant. Mucinous cystadenomas and carcinomas of the ovary are well-established and common tumors. Primary retroperitoneal mucinous cystadenomas and carcinomas are being very rare and histopathogenesis of which is still uncertain. Most pathologists suggest their origin through mucinous metaplasia in a pre-existing mesothelium-lined cyst. An accurate preoperative diagnosis of these tumors is challenging due to lack of established effective diagnostic measures. Hereby presenting a 50-year-old woman, who visited to the hospital with abdominal distension and discomfort since two months. Sonography and computed tomography scans were performed and showed large predominantly cystic lesion arising from right adnexa. Patient underwent exploratory laparotomy for removal of the tumor. Histology and immunohistochemistry revealed primary retroperitoneal mucinous cystadenocarcinoma.

A rare scenario of perforated gastrointestinal stromal tumours of ileum

Gastro-intestinal stromal tumours (GIST) are among the common mesenchymal tumours of the gastro-intestinal (GI) tract. It varies in location and presentation. GIST are reported in the stomach frequently (60-70%), followed by small intestine (20-25%). Mainly GIST manifest typically with bleeding or vague abdominal pain and discomfort. The spontaneous perforation of GIST is very rare. We report case of a middle-age male patient who presented in emergency with pain in right lower abdomen associated with features of peritonism. After clinical evaluation and preliminary radiological investigations, a working diagnosis of perforated appendix was made. Patient was undertaken for emergency surgery. A diagnostic laparoscopy followed by midline laparotomy was done. Intra-operatively, a perforated and necrotic outpouching at antimesenteric border of terminal ileum was found. Histopathological examination of the resected part of ileum revealed compatibility with GIST. It was strongly positive for cluster of differentiation 117 (CD117) and smooth muscle actin. Patient received adjuvant therapy with Imatinib. A complete surgical resection without extensive lymph node sampling is the primary treatment option. As GIST are rare, a high index of suspicion is warranted for diagnosis and appropriate treatment.

Comparison between imprint cytology and frozen sections in intraoperative consultation of ovarian tumours

Background: The ovaries frequently are the site for various primary tumors. Correct intraoperative diagnosis is crucial. The application of imprint cytology is very useful where frozen section facility is not available. The present study is a comparison of imprint cytology and frozen section during intraoperative consultation for various types of benign and malignant ovarian neoplasms in different age groups.Methods: Seventy-six cases of ovarian tumors were examined using both imprint cytology and frozen section and evaluated, taking histopathological report as gold standard. The histopathological diagnoses consisted of benign (54), borderline (9), and malignant (13). The malignant tumors consisted of various types including serous carcinoma, mucinous carcinoma, endometrioid carcinoma, clear cell carcinoma and carcinoid.Results: All 54 benign cases were accurately diagnosed as benign by imprint cytology. With frozen section 53 cases were correctly diagnosed as benign but one case was over diagnosed as borderline. Among 13 malignant cases 11 (84.6%) were correctly diagnosed with both techniques. Borderline tumors were not able to be diagnosed with imprint smear, 3 out of 9 cases were correctly diagnosed with frozen section.Conclusion: When compared with frozen section, imprint cytology is a simple, inexpensive and useful diagnostic tool in intraoperative diagnosis of benign and malignant ovarian tumors. Imprint smear is not useful in borderline tumors where only frozen section is useful. Imprint cytology can be used as an adjunct to frozen section for better diagnosis.

A Review of Adipocytic Tumours, Highlighting the Changing Concepts.

Adipocytic tumours are the most common soft tissue tumours and are frequently encountered in the routine practice. Vast majority of adipocytic tumours are benign lipomatous tumours, but there are other rare heterogenous adipocytic neoplasms which can create diagnostic difficulties. It can vary from a locally recurrent tumour to a highly malignant type carrying a poor prognosis. The classification of adipocytic tumours has evolved in the last few decades due to advances in the understanding of pathogenetic basis that also provides a greater opportunity for the development of new treatment modalities. So, with the use of ancillary diagnostic tests, definite diagnostic criteria have been established, which have been described in WHO 2013 classification and recently a newer terminology have been described, which is Atypical Spindle Cell Lipomatous Tumour and it is considered as a separate entity based on its morphology and molecular studies. Lipomas are the most common benign adipocytic tumours and the less common tumours are lipoblastoma, angiolipoma, spindle cell/pleomorphic lipoma, myolipoma, chondroid lipoma, lipomatosis of nerve and hibernoma. Well differentiated liposarcomas/ atypical lipomatous tumours are locally aggressive adipocytic neoplasms that do not metastasize. More aggressive liposarcomas include myxoid liposarcoma, pleomorphic liposarcoma and dedifferentiated liposarcomas. Within a time period of 6 months we received 362 adipocytic tumours in the department of Pathology, of which 354 were benign and 8 cases were intermediate (locally aggressive) and malignant which includes atypical spindle cell lipomatous tumour, atypical lipomatous tumour/ well differentiated liposarcoma, myxoid liposarcoma and dedifferentiated liposarcoma. The cases of atypical lipomatous tumour and atypical spindle cell lipomatous tumour, its histological and molecular basis will be discussed along with this review of literature. Atypical spindle cell lipomatous tumours are seen usually in the subcutaneous location with a wide anatomic distribution and more commonly seen in the adults of 6th decade. Gross appearance ranges from yellowish to whitish depending on the amount of adipocytic and fibrous differentiation. Microscopic study shows a hypercellular to a hypocellular lesion with mild to moderately atypical spindle cells, adipocytes, lipoblast, and occasional bizarre, hyperchromatic cells. The stroma can be myxoid or collagenous. Mitotic figures are sparse without any tumour necrosis. Rare heterologous differentiation and different growth patterns have been describedincluding solitary fibrous tumour (SFT) like pattern, angiofibroma like pattern, myxoid liposarcoma like pattern and pericytic pattern. Immunohistochemistry studies show variable expression of CD34, S100 and desmin. Molecular studies identified a characteristic loss of nuclear RB gene expression with deletion of 13q14. The indolent behaviour of atypical spindle cell lipomatous tumour, with very low recurrence rate, no risk of dedifferentiation and no reported distant metastasis / death from disease, highlights the importance of distinguishing it from atypical lipomatous tumour/well differentiated liposarcoma, in order to avoid aggressive surgical resections. So atypical spindle cell lipomatous tumour is now considered as an independent entity rather than a morphological variant of atypical lipomatous tumour.

An audit and clinicopathological study of fibroepithelial lesions of the breast at a tertiary care centre and comparison with WHO grading

Background: Mammary fibroepithelial lesions encompass a wide spectrum of tumors ranging from an indolent fibroadenoma to potentially fatal malignant phyllodes tumor (PT). The criteria used for their classification based on morphological assessment are often challenging to apply and there is no consensus as to what constitutes an adequate resection margin. The aims of the study were to study histopathological spectrum of fibroepithelial lesions of breast at tertiary care centre, to stratify and classify various fibroepithelial lesions into fibroadenomas and PT, reclassify all confirmed cases of PT seen in the study period according to standard histopathological WHO criteria.Methods: Records and slides of fibroepithelial lesions of the breast received at the department between January 2016 and August 2019 were retrieved and reviewed.Results: A total 891 fibroepithelial lesions of breast were diagnosed during this duration. Out of these, 826 (92.7%) were fibroadenoma and its variants, 34 (3.8%) cases were of fibroadenomatoid mastopathy and 31(3.5%) were PT. Among all PT, 8 (25.8%) were borderline and 4 (12.9%) were malignant, rest (61.3%) were benign.Conclusions: Fibroepithelial tumours of the breast are a heterogenous group of lesions ranging from fibroadenoma at the benign end of the spectrum to malignant PT. There are overlapping histologic features among various subtypes, and transformation and progression to a more malignant phenotype may also occur. Given the significant clinical differences within various subtypes, accurate pathologic classification is important for appropriate management. Although some immunohistochemical markers may be useful in this differential diagnosis, histomorphology still remains the gold standard.

Histopathological Profile of Skin Adnexal Tumours - A Three Year Experience at a Tertiary Care Centre in Manipur.

Skin adnexal tumours are a large group of benign and malignant tumours that arise from pluripotent stem cells and differentiate toward one or more adnexal structures. So, quite often they may exhibit more than one line of differentiation (hybrid tumours). Moreover, they are uncommon, clinically nonspecific, and remain a diagnostic challenge to surgeons and pathologists alike. The present study is aimed at analysing the histopathological profile of skin adnexal tumours in a hospital based population in Manipur.METHODSThe present study is a retrospective analysis conducted over a period of 3 years from January 2017 to December 2019 in the Department of Pathology, Jawaharlal Institute of Medical Sciences, Manipur, India. Specimens were fixed in formalin, paraffin embedded and stained with haematoxylin and eosin for histopathologic analysis.RESULTSA total of 34 cases of skin adnexal tumours were reported in the Department of Pathology, JNIMS, Imphal, over a period of 3 years out of which 94% (32/34) of the total cases were benign tumours and 5.88% (2/34) were malignant tumours. Tumours with hair follicle differentiation comprised the largest group (44.1%). Pilomatrixoma was the most common tumour followed by Nevus Sebaceous. Both malignant adnexal tumours were sebaceous carcinomas.CONCLUSIONSSkin adnexal tumours are uncommon tumours with most of them being benign tumours and there is a low incidence of malignant tumours. Malignant tumours although rare, need to be diagnosed accurately since they can be aggressive with distant and nodal metastasis. Histopathologic examination remains the gold standard in diagnosing these rare lesions.