RESUMO
In 1924, Darier and Ferrand described Dermatofibrosarcoma Protuberans as a progressive and recurring dermatofibroma. It is a locally aggressive sarcoma originating from dermal and subdermal tissue of the skin. It usually begins as a small plaque that grows over a period and later manifests as multiple small subcutaneous nodules. It is more commonly found in females as compared to males and typically occurs in between 2nd and 5th decades of life. Most frequently involved regions of the body are torso and proximal ends of extremities and very rarely head and neck region is the site of involvement. The mainstay of treatment of this entity is surgery. The rate of recurrence of this disease is very high in about 50% of the cases and it may also express rare distant metastasis. It is a radiosensitive tumour and radiation may play a role in reducing risk of recurrence. We present a case of a 35 years old male with recurrent Dermatofibrosarcoma Protuberans of right parotid gland.
Assuntos
Dermatofibrossarcoma , Recidiva Local de Neoplasia , Glândula Parótida , Neoplasias Parotídeas , Adulto , Biópsia por Agulha Fina/métodos , Dermatofibrossarcoma/metabolismo , Dermatofibrossarcoma/patologia , Dermatofibrossarcoma/cirurgia , Dissecação/métodos , Humanos , Imuno-Histoquímica , Masculino , Margens de Excisão , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/secundário , Recidiva Local de Neoplasia/cirurgia , Glândula Parótida/patologia , Glândula Parótida/cirurgia , Neoplasias Parotídeas/metabolismo , Neoplasias Parotídeas/patologia , Neoplasias Parotídeas/cirurgia , Reoperação/métodos , Resultado do TratamentoRESUMO
BACKGROUND & OBJECTIVE: Pleomorphic xanthoastrocytoma (PXA) is a rare primary WHO Grade II astrocytic tumor comprising of < 1% of all astrocytomas. It is generally benign and slow growing however disease progression and malignant transformation with anaplastic features have been infrequently reported. Our objective was to assess clinicopathological characteristics of this rare tumor at our center. METHODS: A retrospective study was conducted at Aga Khan University Hospital from January 1992 till January 2016. Data was entered on a proforma including patient demographics, clinical features, tumor location, histological features and follow-up, where available. RESULTS: Forty Seven cases of PXA were retrieved during the study period. The mean age was 23.8 years (SD=15.1) and median age was 19 years. The most frequent symptom was head ache (n=31). Male were more frequently affected (n=26). The commonest location was temporal lobe. On microscopic examination, tumors were pleomorphic without mitoses or necrosis, however two cases showed increased mitotic activity, and one case revealed associated gliosarcoma. Follow-up of only 29 cases was available for a period ranging between 2 and 184 months (85 months +/- 56 months). Outcome was good in 27 patients with the last follow up showing no radiographic or clinical evidence of tumor recurrence. CONCLUSIONS: PXA is an infrequent tumor in our population also, with less than 50 cases identified in two decades study period. Due to its rarity and its bizarre histomorphology, it should be diagnosed correctly, as it has got better prognosis than other astrocytic tumors.
RESUMO
OBJECTIVES: To present the clinicopathological experience of Olfactory Neuroblastoma (ONB) with emphasis on histopathological and immunohistochemical features. METHODS: A descriptive cross-sectional study was done on 36 cases of ONB, selected by non-probability purposive sampling. Theses cases of ONB were retrieved and reviewed from surgical pathology database of Aga Khan University Hospital reported between January 1993 and March 2015. RESULTS: Tumor size and age of presentation was wide in range without any distinct bimodal distribution. Nasal cavity was most common site along with involvement of paranasal sinuses. More than 50% cases had Kadish stage A. Microscopically, most cases were Grade-1 and majority showed partial or complete lobular architecture. Neurofibrillary matrix was observed in 2/3(rd) of cases. Among immunohistochemical markers, Neuron Specific Enolase was most frequently expressed. Unusual positive expression of Cytokeratin AE1/AE3 and Cytokeratin CAM5.2 was also seen focally in few cases. CONCLUSION: The ONB has great variability of histological and clinical presentation, and immunohistochemical markers are useful to differentiate from more common small round blue cell tumours of nasal cavity.
RESUMO
AIM: Ovarian steroid cell tumors, not otherwise specified (SCT-NOS) are very rare neoplasms. No large study has been performed in Pakistan to establish the clinicopathological spectrum and immunohistochemical behavior in our region. The purpose of our study was to determine the various clinicopathological and immunohistochemical features of ovarian SCT-NOS along with follow-up in our institution. METHODS: This was a retrospective observational study. The study was conducted in the Section of Histopathology, Aga Khan University Hospital, Karachi, Pakistan. All reported cases of ovarian SCT-NOS occurring during January 1992 to August 2013 were retrieved. The slides were reviewed and patient demographics, and clinical and pathological features were noted with proforma software. SPSS version 19 was used for all analyses. Data is expressed as absolute values and percentages. RESULTS: A total of 12 SCT-NOS (2.3%) out of 528 ovarian sex cord stromal tumors were retrieved. The age range was 3-70 years, with mean of 40.75 years. The tumors ranged 2.5-13 cm in size, with a mean size of 6.1 cm. One patient had bilateral tumors. All of the tumors were positive for inhibin and calretinin. Four tumors were negative for Mic-2 (CD99). In two patients, the tumor recurred. Only one patient who had worse pathological features received adjuvant chemotherapy. CONCLUSION: Steroid cell tumors are very rare ovarian tumors in the Pakistani population, mostly presenting in adulthood. Diverse histological differentials exist so special stains and immunohistochemical stains are needed to distinguish these from other tumors.
Assuntos
Neoplasias Ovarianas/química , Neoplasias Ovarianas/patologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/química , Tumores do Estroma Gonadal e dos Cordões Sexuais/patologia , Antígeno 12E7 , Adulto , Idoso , Antígenos CD/análise , Calbindina 2/análise , Moléculas de Adesão Celular/análise , Pré-Escolar , Feminino , Humanos , Inibinas/análise , Pessoa de Meia-Idade , Paquistão , Recidiva , Estudos Retrospectivos , Carga TumoralRESUMO
Background: Intracranial metastatic deposits due to hepatocellular carcinoma (HCC) are rare. Only a few cases are reported in the literature. These may be more likely to come to clinical attention than extrahepatic metastases in other sites since they often produce symptoms that necessitate neurosurgical intervention. Case Description: We report a case of a 53-year-old male with biopsy-proven intracranial skull base metastasis from HCC as an unusual initial presentation of the disease and review the relevant literature on this entity. Conclusion: Intracranial metastasis of HCC should be included in the differential diagnosis of rapidly growing metastatic lesions in unusual locations, particularly in chronic liver disease and hepatitis B surface antigen-positive or hepatitis C patients.
RESUMO
Background: Ependymomas are usually found in the posterior fossa originating from the fourth ventricle. Primary ependymomas arising from cranial nerves are rare with only a handful of reported cases. Trigeminal neuralgia (TN) is rarely due to space occupying lesions. Case Description: A 20-year-old female presented with TN with a rare presentation of a pure extra-axial ependymoma involving the right trigeminal nerve in the cerebellopontine angle. Conclusion: It is essential to explore the possibility of a mass arising from the trigeminal nerve when investigating the cause of TN.
RESUMO
OBJECTIVE: To find out the clinical and management profile of granulomatous mastitis (GM). STUDY DESIGN: A case. PLACE AND DURATION OF STUDY: This study was carried out at Civil Hospital and Dow University of Health Sciences, Karachi and Tuberculosis Clinic at Bantwa Hospital, Karachi, from January 1999 to January 2009. METHODOLOGY: Histopathologically diagnosed cases of GM were studied. Data included, detailed history, examination, clinical diagnosis, ultrasound, mammogram and fine needle aspiration cytology (FNAC). All patients were initially given antibiotics and corticosteroid for a period of 10 days. All patients were followed for a period of 6 months. Recurrent cases were managed by wide excision and followed for another period of 6 months. Percentage, mean and range values were determined on SPSS version 10. RESULTS: The mean age of the 22 patients was 37.6 years (range=22-52 years). Right breast was affected in 54.5% and left breast in 45.5% patients. Eighteen (82%) were non-lactating. Patients presented clinically with painful breast lump in 54.5%, painless lump in 45.5% and additional discharging sinuses in 18%. Clinical diagnosis was chronic mastitis in 40.9%, tuberculosis of breast in 36.3% and malignancy in 22.7% patients. Mammography showed an ill-defined mass in 45.5% and asymmetrical density in 31.8%. Diagnosis was confirmed in all patients on histopathology. Recurrence was noted in 2 patients, managed by wide excision, and followed for another period of 6 months without evidence of complications. CONCLUSION: GM is an uncommon chronic inflammatory disease of the breast. Usually involving a single non-lactating breast in reproductive age group. It clinically mimics tuberculosis and carcinoma. Mammography remains non-conclusive. Excision and wide excision biopsy are both diagnostic and therapeutic in majority of cases. Treatment includes short course of steroids and antibiotic along with close regular surveillance.
Assuntos
Granuloma/epidemiologia , Mastite/epidemiologia , Adulto , Biópsia por Agulha Fina , Doenças Mamárias/diagnóstico , Neoplasias da Mama/diagnóstico , Diagnóstico Diferencial , Feminino , Granuloma/diagnóstico , Granuloma/patologia , Humanos , Masculino , Mastite/diagnóstico , Mastite/patologia , Pessoa de Meia-Idade , Tuberculose/diagnósticoRESUMO
OBJECTIVE: To compare the postsurgical outcome of laparoscopic and open inguinal varicocelectomy. STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: Department of Surgery, Liaquat University of Medical and Health Sciences, Jamshoro, from July 2003 to June 2007. METHODOLOGY: One hundred seven patients underwent either open inguinal or laparoscopic varicocelectomy. Diagnosis was made by clinical examination and color Doppler scan in doubtful cases. Pre-operative semen analysis was done in all patients. Operative time, postoperative analgesic requirement in number of tablets, hospital stay in days, improvement in semen parameters in subfertile subjects, pain on visual analog score. and postoperative complications were recorded and compared between the two groups. RESULTS: There were 50 patients in each group, with age ranging from 12-50 years (26.9+/-7.67 in open inguinal and 26.2+/-7.08 in laparoscopic group). Average operative time was 34.8+/-7.89 minutes for open inguinal and 43.8+/-8.95 minutes for laparoscopic group. The analgesic requirement was 16.3+/-1.58 tablets in the open inguinal and 11.3+/-2.23 in the laparoscopic group. Postoperative pain was significantly less in the laparoscopic group. There was statistically significant (p < 0.001) improvement in sperm count as well as motility in both groups irrespective of procedure. CONCLUSION: The open inguinal (Ivanissevich) procedure and laparoscopic varicocelectomy had almost equivalent postoperative outcomes regarding improvement in semen parameters and postoperative complications. Open inguinal procedure had a shorter operating time while laparoscopic varicocelectomy had the advantage of less analgesic requirement and short hospital stay. On the whole, open inguinal (loupe magnified) varicocelectomy is an effective procedure where availability and costs of laparoscopic instruments are barriers.
Assuntos
Laparoscopia/métodos , Cordão Espermático/cirurgia , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Varicocele/cirurgia , Adolescente , Adulto , Analgésicos/administração & dosagem , Criança , Humanos , Tempo de Internação/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Medição da Dor , Complicações Pós-Operatórias , Período Pós-Operatório , Recidiva , Reoperação , Sêmen , Resultado do Tratamento , Adulto JovemAssuntos
Encéfalo/patologia , Cordoma/patologia , Proteínas Cromossômicas não Histona/metabolismo , Proteínas de Ligação a DNA/metabolismo , Proteínas Fetais/metabolismo , Proteínas com Domínio T/metabolismo , Fatores de Transcrição/metabolismo , Pré-Escolar , Cordoma/diagnóstico , Feminino , Humanos , Proteína SMARCB1RESUMO
OBJECTIVE: To determine the presentation of breast tuberculosis, diagnostic methods and surgical treatments. STUDY DESIGN: A case series. PLACE AND DURATION OF STUDY: This study was conducted at Dow University of Health Sciences and Tuberculosis Clinic at Bantwa Hospital, Kharadar, Karachi, from April 1999 to March 2007. METHODOLOGY: Clinically diagnosed patients of breast tuberculosis, confirmed by laboratory work-up, were included in this study. Detailed history and examination of both breast and axillae were the primary diagnostic measures. Complete blood counts, ESR, Mantoux test, ultrasound, mammogram, fine needle aspiration cytology, staining for acid-fast bacilli both smear and culture were performed. Core biopsy for lumps more than 5 cm and wide excision biopsy for the lump less than 5 cm were the methods applied. Pre-designed research proforma was filled and descriptive statistics of age, site, side, clinical presentations, investigations were recorded and surgical treatment done. Anti-tuberculosis treatment was given to all patients. RESULTS: Thirty patients were studied with mean age of 28.4 years ranging from 16-48 years. Bilateral breast involvement was seen in 2 patients with 14 cases involving the right and left breast. Lymph node involvement was present in 7. Pulmonary tuberculosis was seen in 4 patients. Multifocal disease was present in 27 patients. The clinical presentation was with lump in 6, discharging sinuses in 14, cold abscess in 8, and non-healing ulcer in 2 patients. There were 5 lactating mothers. Montoux test was positive in 5, AFB smear and culture were positive in 3. Only AFB culture was positive in 4. Five patients required core biopsy for diagnosis of confirmation of lump more than 5 cm and wide excision biopsy was required in 7 patients with lump less than 5 cm. Despite antituberculous treatment, surgical management was required in 21 (70%) cases. CONCLUSION: Tuberculosis of the breast, simulating the carcinoma breast and granulomatous mastitis very closely and are difficult to be differentiated without tissue diagnosis. Surgical management is more often required.
Assuntos
Doenças Mamárias , Tuberculose , Adolescente , Adulto , Doenças Mamárias/diagnóstico , Doenças Mamárias/cirurgia , Feminino , Humanos , Pessoa de Meia-Idade , Tuberculose/diagnóstico , Tuberculose/cirurgiaRESUMO
This prospective study aimed to determine the surgical site infection (SSI) rate and associated risk factors was carried in a general surgical ward at Liaquat University Hospital Jamshoro. A total of 460 patients requiring elective general surgery from July 2005 to June 2006 were included in this study. All four surgical wound categories were included. Primary closure was employed in all cases. Patients were followed up to 30th day postoperatively. All cases were evaluated for postoperative fever, redness, swelling of wound margins and collection of pus. Cultures were taken from all the cases with any of the above finding. Mean +/- SD age of the patients was 38.8 +/- 17.4 years with male to female ratio of 1.5:1. The overall rate of surgical site infection was 13.0%. The rate of wound infection was 5.3% in clean operations, 12.4% in clean-contaminated, 36.3% in contaminated and 40% in dirt-infected cases. Age, use of surgical drain, duration of operation and wound class were significant risk factors for increased surgical site infection (P < 0.05). Postoperative hospital stay was double in cases who had surgical site infection. Sex, haemoglobin level and diabetes were not statistically significant risk factors (P > 0.05). In conclusion, surgical site infection causes considerable morbidity and economic burden. The routine reporting of SSI rates stratified by potential risk factors associated with increased risk of infection is highly recommended.
Assuntos
Procedimentos Cirúrgicos Eletivos/efeitos adversos , Infecção da Ferida Cirúrgica/epidemiologia , Adolescente , Adulto , Fatores Etários , Idoso , Criança , Feminino , Seguimentos , Hospitais Universitários , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Paquistão , Estudos Prospectivos , Fatores de RiscoRESUMO
Invasive aspergillosis (IA) is a disease of the immunocompromised with a predilection for the lungs, although dissemination to all organs is possible. Its diagnosis remains a challenge due to the absence of specific clinical manifestations and laboratory findings. In most cases, diagnosis is eventually made via invasive methods. It carries with it a high mortality due to late diagnosis and delayed treatment. Here, we report a fascinating case of a young, otherwise healthy, immunocompetent patient that presented to us with superior vena cava syndrome and a mediastinal mass. It was anticipated that a malignancy would be found on further workup but, in fact, what was eventually discovered was a case of IA. Our report accentuates the significance of including IA as a differential while diagnosing a mediastinal mass in an immunocompetent host as patient outcome is determined by timely diagnosis and treatment.
Assuntos
Aspergilose/diagnóstico , Doenças do Mediastino/diagnóstico , Síndrome da Veia Cava Superior/diagnóstico , Adulto , Antifúngicos/uso terapêutico , Aspergilose/tratamento farmacológico , Aspergillus flavus/isolamento & purificação , Diagnóstico Diferencial , Humanos , Imunocompetência , Masculino , Doenças do Mediastino/tratamento farmacológico , Doenças do Mediastino/microbiologia , MediastinoRESUMO
Epidermoid cyst of the presacral space is a rare congenital lesion of ectodermal origin. Presacral epidermoid cysts have been previously reported in women, however are extremely rare in males. We report a case of presacral epidermoid cyst in a 55-year-old male who presented to our emergency department with acute urinary retention and history of chronic constipation. A non-contrast computed tomography scan was performed with suspicion of urolithiasis, which revealed a well circumscribed low attenuation presacral mass. Magnetic resonance imaging (MRI) of the pelvis was subsequently performed to further characterize the lesion. The mass was returning hypointense T1 and hyperintense T2 signals with few foci of T2 hypointensities. There was no post-contrast enhancement; however the lesion was showing diffusion restriction, appearing hyperintense on diffusion weighted imaging (DWI) and hypointense on the corresponding apparent diffusion coefficient map. These imaging features were consistent with an epidermoid cyst. Laparotomy with complete surgical excision of the cyst and preservation of the adjacent structures was performed. The histopathology confirmed the diagnosis. This case highlights the importance of MRI with additional sequences of diffusion weighted imaging which can be helpful to differentiate, to a good degree of confidence, among different pelvic tumors, therefore obviating the need of biopsy before surgery.
RESUMO
A thorough analysis of the photophysical properties involved in electronic transitions in excitation-emission spectra of xylene isomers has been carried out using the time-dependent density functional theory (PBEPBE/6-31 + G(d,p)) method. For the first time a structural and spectroscopic investigation to distinguish isomers of xylene, a widespread priority pollutant, was conducted experimentally and theoretically. The fluorescence properties of xylene isomers (sole and mixture (binary and ternary)) in water were studied. The fluorescence peak intensities of xylenes were linearly correlated to concentration, in the order of p-xylene > o-xylene > m-xylene at an excitation/emission wavelength (ex/em) of 260â nm/285â nm for o-, m-xylene and ex/em 265â nm/290â nm for p-xylene at the same concentration. The theoretical excitation/emission wavelengths were at ex/em 247â nm/267â nm, 248â nm/269â nm and 251â nm/307â nm for o-, m- and p-xylene, respectively. The vertical excitation and emission state energies of p-xylene (ex/em 4.94â eV/4.03â eV) were lower and the internal conversion energy difference (0.90â eV) was higher than those of m-xylene (ex/em 5.00â eV/4.60â eV) (0.4â eV) and o-xylene (ex/em 5.02â eV/4.64â eV) (0.377â eV). The order of theoretical emission and oscillator strength (0.0187 > 0.0175 > 0.0339) for p-xylene > o-xylene > m-xylene was observed to be in agreement with the experimental fluorescence intensities. These findings provide a novel fast method to distinguish isomers based on their photophysical properties.
RESUMO
BACKGROUND: Advancing age with its associated co-morbidities increases the likelihood of postoperative complications as well as conversion rate during laparoscopic cholecystectomy. Recent studies have also questioned efficacy of this procedure in geriatric patients. The present study assesses the safety and applicability of laparoscopic cholecystectomy in geriatric patients. The objective of the present study was to asses safety and applicability of laparoscopic cholecystectomy in the elderly patients of 65 years and above. METHODS: This is a prospective analysis of 173 patients, over 65 years of age, who underwent laparoscopic cholecystectomy from December 2002 to November 2006 at Liaquat University Hospital, Jamshoro. Patients presenting with complicated and uncomplicated gallstone disease were included in the study population and all of them were operated laparoscopically. The data included demographic details, co-morbidities, underlying biliary pathology, indications for surgery, operative and postoperative complications, morbidity and mortality, and hospital stay. The statistical analysis of the data performed on SPSS version 10. RESULTS: Laparoscopic cholecystectomy undertaken in 173 elderly patients with a mean age of 69.72 years, out of whom 52 (30.05%) were males and 121 (69.94%) were females. Co-morbid conditions were identified in 53.17% (n = 92) patients and included hypertension in 38 patients (21.96%), Diabetes Mellitus in 23 patients (13.29%), COPD in 19 (10.98%) patients, Coronary artery disease in 9 (5.20%) and cardiac arrhythmias in 3 (1.73%) patients. Indications for surgery included simple biliary colic in majority of patients (69.94%) and complicated stone disease in 52 (30.05%) subjects. There were 37 (21.38%) emergency laparoscopic cholecystectomies and 136 (78.61%) patients were operated electively. Mean operative time was 100 minutes with a SD 29.03. Fourteen (8.09%) patients required conversion to OC (Open Cholecystectomy) due to various reasons. Mean hospital stay was 6.28 days. Overall 23 (13.29%) patients developed postoperative complications. One patient died of acute MI on 2nd postoperative day. CONCLUSION: There is no undue risk in laparoscopic cholecystectomy in the elderly population and the procedure can be regarded as safe as in patients below 65 years of age.
Assuntos
Colecistectomia Laparoscópica , Doenças da Vesícula Biliar/cirurgia , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Feminino , Doenças da Vesícula Biliar/complicações , Nível de Saúde , Hospitais Universitários , Humanos , Masculino , Paquistão , Estudos ProspectivosRESUMO
OBJECTIVE: To find out the safety profile of laparoscopic cholecystectomy in empyema of gallbladder. BACKGROUND: Empyema of gall bladder is a severe form of acute cholecystitis with superadded suppuration. It has been considered a contraindication for the laparoscopic cholecystectomy (LC) because of fear of life-threatening complications. This study aimed to determine the safety and feasibility of LC in empyema of gallbladder. MATERIALS AND METHODS: LC was attempted in 67 patients of empyema of gallbladder within 24h. However in few cases there was a delay because of reluctance for surgery or delay in giving consent etc. The procedure was performed by standard four-port technique with few changes made to facilitate dissection according to situation. RESULTS: Between April 2003 to June 2006, 970 LC performed for gallstone disease at surgical unit-1 of LUMHS by the same surgical team. Among these, 67 (6.90%) patients were diagnosed to have empyema gall bladder. LC successfully completed in 54 (80.59%) patients. In 13 (19.40%) patients the procedure was converted to open cholecystectomy (OC) due to various operative difficulties of which the most serious injuries included bleeding from cystic artery (four cases), common bile duct injury (two cases) and duodenal injury in one case. Maximum operating time was up to 160 minutes (one case). Postoperative complications occurred in 10 (18.51%) successfully operated patients. Maximum patients (n=45, 83.33%) were discharged in 48-96 hours while three patients were discharged after two weeks. CONCLUSION: Laparoscopic cholecystectomy can be performed in empyema of gallbladder keeping in mind a slightly increased risk of complications even in the best hands. However, the experience of the surgeon plays a key role in the overall outcome.
RESUMO
Background: Atypical teratoid/ rhabdoid tumor (AT/RT) is a rare aggressive embryonal central nervous system (CNS) tumor of infancy and early childhood. Majority of the cases arise in the posterior fossa, and remaining in the cerebrum. Aims: To analyze the clinicopathologic features of AT/RT on a cohort of cases. Materials and methods: All reported cases of AT/RT at the Department of Pathology and Laboratory Medicine, Aga Khan University Hospital (AKUH) from 2007 to 2016 were reviewed for clinical and pathological features. Immunohistochemical stain for INI-1 was performed in all 11 cases. Follow up was obtained. Results: A total of 11 cases were identified. Seven patients were males and 4 were females. The ages ranged from 1 month to 48 months (mean 26.6 months). Six tumors were located in the cerebrum and 3 in the posterior fossa. Exact Location was not known in 2 cases. Histologically, rhabdoid cells were present in sheets in variable proportions in five cases, Medulloblastoma and PNET like areas were seen in 2 cases each. Immunohistochemical stains EMA (10/10), vimentin (7/7), CKAE1/AE3 (8/9), and CD99 (3/4), GFAP (6/10), ASMA (3/4) and synaptophysin (3/4) were positive in varying proportions while desmin was negative in all 6 cases in which it was performed. All 11 tumors lacked immunoreactivity for INI-1 protein. Four patients died of disease with a follow up ranging from 5 to 24 months. Conclusions: AT/RT is a rare highly aggressive embryonal tumor of CNS. A male predominance was noted in our series. We report the first and largest series from Pakistan.
RESUMO
The 2016 update of the WHO Classification of Tumours of the Central Nervous System has redefined a number of tumors. Embryonal tumor with multilayered rosettes, C19MC-altered is one such tumor entity which has been newly defined on the basis of a characteristic molecular alteration. We report, to our knowledge, the first case of this rare pediatric brain neoplasm in the Pakistani population. An 8-month-old girl was presented with vomiting and left-sided ptosis, and magnetic resonance imaging scan showed a cerebellar tumor. Histologically, a highly cellular population of primitive cells was seen alternating with hypocellular neuropil-rich regions containing multilayered true rosettes and cells with glial and neuronal differentiation. Amplification of 19q13. 42 chromosome region on fluorescence in situ hybridization analysis confirmed the diagnosis. Post-operative radiological examination revealed widespread central nervous system involvement. Adjuvant treatment was not offered due to complications. Patient expired a week after diagnosis.
RESUMO
BACKGROUND: Central neurocytomas are rare neuronal neoplasms with a favorable prognosis. They are typically located in the lateral ventricles of the brain and mostly histologically correspond to WHO grade II with a Mib 1 labelling index of <2%. Similar tumors located in the cerebral hemispheres and spinal cord, for example, are called "extraventricular neurocytomas". A few tumors histologically show atypia, mitoses, vascular proliferation and/or necrosis and a Mib 1 index >2 % and are designated as "atypical neurocytomas. AIM: The aim of our study was to describe the common as well as unusual morphologic features and the role of various immunohistochemical stains in the diagnosis of these rare tumors. MATERIALS AND METHODS: We retrieved and reviewed 35 cases diagnosed between 2001 and 2015. RESULTS: Sixty percent of patients were males, and the mean age was 26 years. 31 cases (88.6%) were intraventricular and 4(11.4%) were extraventricular. Histologically, 6 cases (17.1%) were compatible with "atypical neurocytomas". All cases showed the classic morphology comprising nests and sheets of uniform, round cells with uniform round to oval nuclei with finely speckled chromatin and perinuclear cytoplasmic clearing (halos). All cases also showed delicate, fibrillary, neuropil-like matrices. Other common histologic features included capillary-sized blood vessels in a branching pattern in 57.1%, foci of calcification in 34.3% and perivascular pseudorosettes in 20%. Rare findings included Homer- Wright or true rosettes in 8.6% and ganglioid cells in 2.9%. Synaptophysin was the most consistent and valuable marker, being positive in almost all cases. GFAP positivity in tumor cells was seen in 25.7% of cases. Follow up was available in 13 patients. Of these 9 had histologically typical and 4 had atypical tumors. Only 1 (with an atypical neurocytoma) died, probably due to complications of surgery within one month, while 12 (including 3 with atypical neurocytomas) remained alive. Recurrence developed in 1 of these 12 patients (histologically consistent with typical morphology) almost 9 years after surgery. Only 4 patients, including 2 with atypical tumors, received postoperative radiotherapy, all with surgery in 2010 or later. Overall, prognosis was excellent with prolonged, recurrence free survival and most patients, even without receiving radiation therapy, were alive and well for many years, even a decade or more after surgery, without developing any recurrence, indicating the benign nature of these neoplasms.
Assuntos
Neoplasias Encefálicas/patologia , Neurocitoma/patologia , Adolescente , Adulto , Biomarcadores Tumorais/metabolismo , Neoplasias Encefálicas/epidemiologia , Neoplasias Encefálicas/metabolismo , Criança , Feminino , Seguimentos , Humanos , Técnicas Imunoenzimáticas , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neurocitoma/epidemiologia , Neurocitoma/metabolismo , Paquistão/epidemiologia , Prognóstico , Adulto JovemRESUMO
CONTEXT: There are no recent authoritative data about incidence and prevalence of various types of cancers in Pakistan. AIM: To determine the frequency of malignant tumors seen in our practice and provide a foundation for building a comprehensive cancer care strategy. MATERIALS AND METHODS: 10,000 successive cases of solid malignant tumors reported in 2014 were included. All cases had formalin fixed, paraffin embedded specimens available and diagnosis was based on histological examination of H and E stained slides plus ancillary studies at the Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi. The latest WHO classifications were used along with the latest CAP protocols for reporting and the most updated TNM staging. RESULTS: There were 9,492 (94.9%) primary tumors while 508 (5.1%) were metastatic. Some 5,153 (51.5%) were diagnosed in females and 4,847 (48.5%) in males. The commonest malignant tumors in females were breast (32%), esophagus (7%), lymphomas (6.8%), oral cavity (6.7%) and ovary (4.8%), while in males they were oral cavity (13.9%), lymphomas (12.8%), colorectum (7.9%), stomach (6.9%) and esophagus (6.6%). Malignant tumors were most common in the 5th, 6th and 7th decades. About 8% were seen under 20 years of age. CONCLUSIONS: Oral cavity and gastrointestinal cancers continue to be extremely common in both genders. Breast and esophageal cancers are prevalent in females. Lung and prostate cancer are less common than in the west. Ovarian cancer was very common but cervix cancer was less so.