Capacity building for pediatric neuro-oncology in Pakistan- a project by my child matters program of Foundation S.

Introduction: Initiated in June 2019, this collaborative effort involved 15 public and private sector hospitals in Pakistan. The primary objective was to enhance the capacity for pediatric neuro-oncology (PNO) care, supported by a My Child Matters/Foundation S grant. Methods: We aimed to establish and operate Multidisciplinary Tumor Boards (MTBs) on a national scale, covering 76% of the population (185.7 million people). In response to the COVID-19 pandemic, MTBs transitioned to videoconferencing. Fifteen hospitals with essential infrastructure participated, holding monthly sessions addressing diagnostic and treatment challenges. Patient cases were anonymized for confidentiality. Educational initiatives, originally planned as in-person events, shifted to a virtual format, enabling continued implementation and collaboration despite pandemic constraints. Results: A total of 124 meetings were conducted, addressing 545 cases. To augment knowledge, awareness, and expertise, over 40 longitudinal lectures were organized for healthcare professionals engaged in PNO care. Additionally, two symposia with international collaborators and keynote speakers were also held to raise national awareness. The project achieved significant milestones, including the development of standardized national treatment protocols for low-grade glioma, medulloblastoma, and high-grade glioma. Further protocols are currently under development. Notably, Pakistan's first pediatric neuro-oncology fellowship program was launched, producing two graduates and increasing the number of trained pediatric neuro-oncologists in the country to three. Discussion: The initiative exemplifies the potential for capacity building in PNO within low-middle income countries. Success is attributed to intra-national twinning programs, emphasizing collaborative efforts. Efforts are underway to establish a national case registry for PNO, ensuring a comprehensive and organized approach to monitoring and managing cases. This collaborative initiative, supported by the My Child Matters/Foundation S grant, showcases the success of capacity building in pediatric neuro-oncology in low-middle income countries. The establishment of treatment protocols, fellowship programs, and regional tumor boards highlights the potential for sustainable improvements in PNO care.

Skull base metastasis as initial presentation of hepatocellular carcinoma.

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.

Multifocal oligodendroglioma with callosal and brainstem involvement.

Background: Oligodendrogliomas are generally low-grade glial neoplasms commonly occurring in a cortical or subcortical location and frequently contain coarse calcifications. Tumors with 1p and 19q codeletions behave atypically and are more likely to have ill-defined margins and tend to have calcification. Very rarely, diffuse pattern and gliomatosis type of infiltrative nature of oligodendrogliomas have been described in sporadic case reports. Case Description: In this article, we present a case of a 31-year-old male who had diffuse multifocal oligodendroglioma with rare features of extensive callosal and brainstem involvement on imaging. Conclusion: Rare cases of oligodendrocytic gliomatosis cerebri or oligodendrogliomatosis with diffuse white matter spread of these tumors usually lead to a detrimental course of neurological status and a poor prognosis in these patients.

Giant Central Nervous System Aspergilloma Mimicking Butterfly Neoplasm of the Corpus Callosum.

Radiological presentation of central nervous system (CNS) aspergillosis is variable and depends on the immune status of the patients. Typical features of meningoencephalitis, infarction, abscess, and mycotic aneurysms commonly occur in immunocompromised patients. A rare mass-like or tumoral form of cerebral aspergillosis has been described mostly in immunocompetent patients which results in a diagnostic dilemma, thus potentially causing a delay in the management. We present a case of a large CNS aspergilloma mimicking an infiltrative callosal neoplasm in a young immunocompetent patient. Careful evaluation of imaging features, anatomical location, enhancement pattern, concomitant sinonasal and orbital extension, and angio-aggressive nature of the mass lesion with a high index of suspicion can help diagnose CNS aspergillosis in such patients.

Assessment of the normal pediatric pineal gland - MR findings of a single centre; A retrospective study.

OBJECTIVE: To analyse the size and morphology of the normal pineal gland in the paediatric age group using magnetic resonance imaging. METHODS: The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data of patients aged <18 years who had magnetic resonance imaging done between 2007 and 2017. Pineal size was estimated measuring its largest anteroposterior and supero-inferior diameters on sagittal T2-weighted sequences on any slice with maximal diameter. The width of pineal gland was measured on axial or coronal T2 sequences. The volume, morphology and enhancement patterns of the gland were also assessed. Data was analysed using SPSS 21. RESULTS: Of the 200 patients, 116(58%) were males and 84(42%) were females. The overall mean age was 7.8±5.5 years. The mean anteroposterior dimension was 4.85±1.46mm, height 3.31±0.95mm, width 4.16±1.19, and volume 39.54±38.12 mm3. Mean size of cyst was 2.77±1.4mm (range: 1.2-7.5mm). No significant differences in the pattern of enhancement were found in different age groups (p>0.05). CONCLUSIONS: Comprehensive knowledge of the size of the typical pineal organ is useful for radiologists in the identification of pineal gland anomalies and the exclusion of neoplastic lesions.

The many faces of glioblastoma: Pictorial review of atypical imaging features.

Glioblastoma is an aggressive primary central nervous system tumour that usually has a poor prognosis. Generally, the typical imaging features are easily recognisable, but the behaviour of glioblastoma multiforme (GBM) can often be unusual. Several variations and heterogeneity in GBM appearance have been known to occur. In this pictorial essay, we present cases of pathologically confirmed GBM that illustrate unusual locations and atypical features on neuroimaging, and review the relevant literature. Even innocuous-looking foci, cystic lesions, meningeal-based pathology, intraventricular and infra-tentorial masses, multifocal/multicentric lesions and spinal cord abnormalities may represent GBM. We aim to highlight the atypical characteristics of glioblastoma, clarify their importance and list the potential mimickers. Although a definitive diagnosis in these rare cases of GBM warrants histopathological confirmation, an overview of the many imaging aspects may help make an early diagnosis.

Interobserver Reliability of the RENAL Nephrometry Scoring System: Experience From a Developing Nation.

Introduction The RENAL (radius, exophytic/endophytic properties, nearness of tumor to the collecting system or sinus in millimeters, anterior/posterior, location relative to polar lines) nephrometry score (RENAL-NS) has been described as a structured and quantifiable method to describe a renal tumor's relevant anatomic features as they relate to the complexity of the lesion. We aim to evaluate a tumor's RENAL-NS and to assess the reproducibility of the score among different observers. Methods This retrospective study included 49 patients diagnosed with renal cell carcinoma (RCC) who had complete computed tomography (CT) data, RENAL-NS, and histopathology results. All patients underwent renal surgery/intervention at our center between January 2008 and December 2018. The radius of the lesion, exophytic/endophytic properties, nearness to the collecting system, anterior or posterior description, and location relative to the polar lines was used to calculate the score. Tumor complexity was graded as low, intermediate, or high. Two body imaging radiologists evaluated the data independently. Results Interobserver agreement for each of the RENAL-NS parameters, respectively, and overall complexity was calculated. The total agreement was 82%, 51%, 84%, 69%, 73%, and 90%, corresponding to Kappa values of 0.72, 0.33, 0.44, 0.49, 0.58, and 0.83, respectively. The radius, nearness to the collecting system, and total complexity showed the best agreement. Exophytic properties of the lesion showed the least agreement. For cases that were discordant in terms of the final score, no major implications in surgical planning were observed. Conclusion The results of this study show that the RENAL-NS is a useful tool to assess the anatomical features of renal tumors and it is easily reproducible, even for less experienced radiologists in a developing nation.

Spontaneous Uterine Rupture Secondary to Morbidly Adherent Placenta in an Unscarred Uterus.

We report a case of spontaneous uterine rupture in a primigravida with an unscarred uterus, which was secondary to morbidly adherent placenta proven on surgery and histology. Although rare, uterine rupture should be considered as a differential diagnosis of acute abdominal pain in pregnancies, especially when associated with free fluid, even with the absence of vaginal bleeding. Abnormal placentation is associated with spontaneous antepartum uterine rupture even in early pregnancy. Most cases in the literature have advocated emergency hysterectomy to arrest life-threatening hemorrhage.

Utility of Ultrasound and Mammography in Detection of Negative Axillary Nodal Metastasis in Breast Cancer.

Objective The status of axillary lymph nodes is one of the most important prognostic factors in patients with breast cancer. A precise noninvasive evaluation of axillary lymph node status preoperatively, although challenging, is vital for optimization of the treatment plan for patients. The objective of our study was to assess the utility of ultrasound and mammography in detecting the absence of axillary lymph nodal metastasis in patients of breast cancer, taking histopathology as gold standard. Methods A cross-sectional study was conducted in the Department of Radiology, Aga Khan University Hospital, Karachi. All female patients between 20 and 95 years of age with a known diagnosis of breast cancer with mammographic and ultrasound imaging done at our institute were included. Patients with abnormal lymph nodes on mammography or on ultrasound, patients already operated for breast cancer, patients who already underwent axillary lymph node dissection and those whose histopathology reports were not available or who did not undergo surgery were excluded. Results A total of 262 women with breast carcinoma who had both ultrasound and mammography done and also had surgery performed at our institution were included. At final surgical pathology, a total of 45 of the 262 patients (17.2%) with breast carcinoma had one or more positive lymph nodes. Out of the total 262 patients, 217 patients were found to be true negatives as they had absent axillary nodal metastasis on imaging as well as on histopathology. In all, 45 out of 262 patients were found to be false negatives as they had absent axillary nodal metastasis on imaging; however, they were found to be positive for metastasis on histopathology. The negative predictive value was 82.8%. Patient age was considered as a factor that may influence the outcome of results; the patients were stratified into age ranges seven groups with the age range of 10 years, ranging from 26 to 95 years. Chi-square test showed a p-value of 0.148, which showed no significant difference in the effect of age on diagnosing the absence of metastasis by ultrasound and mammography. Conclusion Our study shows that ultrasound and mammography even when used in combination cannot safely exclude axillary metastasis and thus cannot eliminate the need for sentinel node biopsy.

Image-guided Percutaneous Radiofrequency Ablation for Osteoid Osteoma: Experience from a Developing Nation.

Objective The purpose of this study is to report our experience in using image-guided percutaneous radiofrequency ablation (RFA) for the treatment of osteoid osteoma (OO) and the subsequent duration of pain relief over a period of about six years (May 2013-March 2019; 70 months) at a tertiary-care hospital in a developing nation. Methods A retrospective study was performed at the radiology department of Aga Khan University, Karachi, Pakistan. All patients who had undergone image-guided percutaneous RFA for OO between May 2013-March 2019 were included. All cases had been performed with CT-guidance under general anesthesia, with an additional local anesthesia injection also administered to the patients. A soloist needle had been used for RFA. The primary success rates, complications, symptom-free intervals, and follow-ups were evaluated. Results In total, 15 patients (11 males, 4 females) of a mean age of 13.93 years (range: 5-25 years; median age: 14.5 years) with OO underwent image-guided percutaneous RFA during a period of 70 months. Eleven lesions were located in the femur, three in the tibia, and one in the humerus. The mean nidus size was 8.1 x 5.73 mm [range: (4.9-11.5) x (3.8-9.1) mm]. All patients were successfully treated and experienced resolution of pain in 2.36 months (range: 1-4 months). During the follow-up period (range: 3-40 months; mean: 13.85 months; median: nine months), none of the patients experienced any relapse or persistent symptoms. No major complications were reported. Conclusion Image-guided percutaneous RFA is a minimally invasive and safe treatment option with high efficiency and a high rate of technical success for the treatment of OO. The risk of recurrence is remote with all patients achieving independent recovery.

Bilateral Pulmonary Artery Mycotic Aneurysms in a Female with Infective Endocarditis.

We present a case of a young female who had large bilateral pulmonary artery aneurysms and was found to have infective endocarditis on echocardiography. Endovascular treatment was sought; however, was not possible due to severe sepsis and other associated complications. Subsequent serial imaging revealed the rapid enlargement of one of the aneurysms and possible rupture which proved to be fatal. Although pulmonary artery aneurysms are a rare vascular anomaly, they are seen in a wide variety of conditions, such as congenital heart disease, infection, trauma, pulmonary hypertension, cystic medial necrosis, and generalized vasculitis. This case highlights the consequences of mycotic aneurysms and emphasizes the importance of prompt diagnosis. Early angioembolization and/or surgical repair is often essential to avoid death from rupture of the aneurysm.

Cholecystocolonic Fistula.

Cholecystocolonic fistula (CCF) is a rare complication of gallstone disease with a variable clinical presentation. It is difficult to diagnose CCF pre-operatively despite modern diagnostic and imaging modalities as they are often asymptomatic or incidentally discovered, often peri-operatively. However, management of this uncommon yet important finding is not very well described in the literature. The most common fistula is the cholecystoduodenal fistula, followed by the cholecystocolonic fistula; the cholecystogastric fistula is reportedly the least commonly reported. We report our experience with three cases of cholecystocolonic fistula discovered on imaging which were subsequently confirmed through surgery.

Polypoid Adenomyoma of the Uterus.

Polypoid adenomyoma is a rare uterine endometrial polypoid tumor of mixed epithelial and mesenchymal origin. Although the clinical and pathologic features of polypoid adenomyomas have been described extensively, imaging findings for these tumors have not been frequently reported in the literature. On imaging, their features may be confused with prolapsed leiomyomas or malignancy. Hemorrhagic cystic spaces in a prolapsed uterine tumor within the vagina should raise consideration of a diagnosis of polypoid adenomyoma. Such blood-containing cystic spaces would be unusual findings in leiomyomas and malignancy. Diagnosing polypoid adenomyoma is vital because it can potentially be managed by hysteroscopic resection, unlike an ordinary form of adenomyosis.

Usefulness of Delayed Films of Contrast Enema for Detecting Hirschsprung's Disease.

Background Contrast enema (CE) in Hirschsprung's disease (HD) provides a road map to surgeons by ascertaining the transition zone (TZ) and helps in pre-surgical planning. In our institute, we use CE as the initial investigation for HD and carry on till the whole colon is fully distended, followed by a 24-hour abdominal film which is also a part of the international protocol. The main aim of this study was to evaluate the usefulness of this 24-hour delayed film in detecting HD, compare it with gold-standard biopsy results, and to evaluate other imaging features of contrast enema for diagnosis of HD in our tertiary-care hospital in Pakistan.  Methods This retrospective study was conducted at the Department of Radiology, Aga Khan University Hospital, Karachi. Records of pediatric patients referred for radiological evaluation of symptoms and signs suspicious of HD during the years 2007-2017 were reviewed. A delayed film was labeled positive if the contrast was not completely evacuated when the residual contrast was present till transverse colon and not beyond. Specificity and sensitivity along with positive and negative predictive values were calculated for each finding according to rectal biopsy, taken as the gold standard. Results In all, 82 patients met the inclusion criteria out of 111 cases, as they had both biopsy results and delayed 24-hour films. HD was confirmed using rectal biopsy in 56 (43 patients were males and 13 were females) of 82 cases. The most sensitive radiological finding was the transition zone with a sensitivity of 91.07%. The rectosigmoid index was the second most common finding on contrast enema with a sensitivity and specificity of 91.07% and 83.93%, respectively. In all, 59% patients had a positive delayed 24-hour film and were confirmed with having HD on biopsy. The sensitivity, specificity, and positive predictive value of delay in contrast evacuation after 24 hours in our study was 81.25%, 90.91%, and 97.50% respectively. Conclusion Contrast enema examinations along with the 24-hour delayed film with mid transverse colon cut-off are optimal for initially investigating HD in a developing nation, and our results show that it correlates well with biopsy. However, rectal biopsy still remains the gold standard for diagnosis.

Lumbar Artery Pseudoaneurysm Following Renal Biopsy.

Lumbar artery pseudoaneurysms have previously been described as rare iatrogenic complications following percutaneous interventional procedures involving the flanks. We describe a case of a 71-year-old man who became unstable and dropped 3 grams of hemoglobin within 24 hours following renal biopsy. A post-biopsy hemorrhage was suspected, and a pseudoaneurysm of his second right lumbar (L2) artery was found on computed tomography angiogram (CTA). Successful coil embolization was performed in the right L2 artery. This case discusses the diagnostic and therapeutic challenges of this unusual complication as well as the anatomical and technical factors involved in the embolization of the lumbar arteries.

Metachronous renal Ewing sarcoma/primitive neuroectodermal tumour in a survivor of Burkitt lymphoma.

We present a case of a 14-year-old girl who was diagnosed with Burkitt lymphoma in 2014. She was managed with chemotherapy and remained in remission for 3 years. On her surveillance imaging in 2017, a left-sided renal neoplastic mass was incidentally discovered. She underwent nephrectomy and pathology of the resected specimen revealed small cell tumour of the kidney with features favouring renal Ewing sarcoma/primitive neuroectodermal tumour. Molecular genetic analysis by fluorescence in situ hybridisation was performed which showed translocation of 22q12, thereby confirming the diagnosis. This is a rare secondary malignancy and an unusual association. This case highlights the importance and diagnostic dilemmas of rare secondary tumours in patients with such haematological malignancies and discusses its possible pathogenetic aspects.

Torsion of Ovarian Dysgerminoma in a Child: Role of Computed Tomography.

Dysgerminomas are malignant germ cell tumors of the ovary that most commonly occur in the adolescent population. Ovarian dysgerminoma presenting with complications like torsion is a rare entity in the pediatric age group. Cross-sectional imaging plays a crucial role in diagnosis, tumor staging before surgical resection, and for planning adjuvant chemotherapy. We report a case of a nine-year-old female who presented to the emergency room (ER) with abdominal distention and abdominal pain. Computed tomography scan revealed a large right-sided pelvic mass with areas of low attenuation, speckled calcification, peritumoral free fluid, and a twisted vascular pedicle that was likely originating from the left adnexa. The right ovary was normal in appearance. Suspicion of a left-sided ovarian tumor with torsion was raised, which was later confirmed on surgery and histopathology of the resected specimen.

Hepatic, Periportal, Retroperitoneal, and Mesenteric Neurofibromatosis in von Recklinghausen's Disease.

We present a rare case of histologically proven neurofibromatosis of the liver, hepatic hilum, retroperitoneum, and mesentery. An adult male who had been diagnosed with neurofibromatosis (NF) type 1 underwent a computed tomography (CT) scan for abdominal pain and vomiting. The CT scan showed a large low-attenuating lesion in the region of porta hepatis which was infiltrating along portal tracts into the liver, encasing the major vessels, and extending into the retroperitoneum and mesentery. Based on the radiological findings, a differential diagnosis of plexiform neurofibroma was given, although sarcomatous transformation could not have been entirely excluded from imaging alone. The tumor was subsequently biopsied, and the histopathological analysis confirmed the diagnosis of neurofibroma. This case highlights the importance and diagnostic dilemmas in the presence of this tumor at atypical locations in this disease spectrum.

A Rare Case of Presacral Epidermoid Cyst in an Adult Male: Emphasis on Diffusion Weighted Magnetic Resonance Sequences in Preoperative Imaging.

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.