Isolated High-Grade Malignancy of the Spinal Cord Presenting as Longitudinally Extensive Transverse Myelitis

This article characterizes 2 cases of longitudinally extensive transverse myelitis (LETM) that did not respond to immunotherapy and were diagnosed by biopsy as primary central nervous system (CNS) malignancies. Diffuse H3 K27M-mutant glioma is a recently described entity with very few cases of isolated spinal disease described in adults. Primitive neuroectodermal tumor is similarly uncommon in the spinal cord. Malignancies should be considered in patients who fail to improve with immunomodulatory therapy. We believe the experiences of our center will raise awareness about that point, broaden the existing understanding of the diagnostic approach to LETM, and highlight the need for additional studies.

Predictors of laboratory spontaneous tumour lysis syndrome in children with high-grade tumours in Uganda

High-grade malignancy is endemic in sub-Saharan Africa and is prone to the spontaneous tumour lysis syndrome. However, data on spontaneous tumour lysis syndrome remain scanty in our setting. We sought to determine the prevalence and factors associated with laboratory spontaneous tumour lysis syndrome in children in Uganda. We conducted a cross-sectional study among children <18 years old with histologically confirmed high-grade malignancy between October 2013 and April 2014. Laboratory spontaneous tumour lysis syndrome was defined as the presence of ≥2 of each of hyperkalaemia, hypocalcaemia, hyperuricaemia and hyperphosphatemia prior to administration of chemotherapy when alternative diagnoses had been excluded. A p < 0.05 was considered statistically significant. Of 108 children, of median age 7.7 years, where boys outnumbered girls 2:1, high-grade, malignancy included Burkitt’s lymphoma, acute lymphoblastic leukaemia, non-Hodgkin’s lymphoma, acute myeloid leukaemia and Burkitt’s leukaemia, with 14 suffering with laboratory spontaneous tumour lysis syndrome. Hypocalcaemia was its most common electrolyte imbalance; and four children died prior to commencement of chemotherapy. Bulky disease, lactate dehydrogenase levels ≥500 iu/l and serum creatinine levels >1.2 mg/dl were associated with laboratory spontaneous tumour lysis syndrome. However, only bulky disease was significantly predictive of laboratory spontaneous tumour lysis syndrome. Such children would benefit from routine screening.

Olfactory Preservation in Craniofacial Resection of Tumor Invading Hemianterior Skull Base: Operative Video

In traditional craniofacial resection of tumors invading the anterior skull base, the bilateral olfactory apparatus is resected. Recently, transnasal endoscopy has been used for olfactory preservation in resections of unilateral low-grade malignancies. However, for tumors that invade the orbita or for high-grade malignancies, the transnasal endoscopic skull base surgery has been controversial. This video demonstrates the surgical techniques of olfactory preservation during craniofacial resection of a high-grade malignancy invading the hemianterior skull base and orbita.We present the case of a 32-year-old woman with osteosarcoma in the right ethmoid sinus. The tumor invaded the ipsilateral cribriform plate, dura menta, and orbital periosteum; however, the nasal septum and crista galli were intact (Fig. 1A, B). Because the tumor was a high-grade malignancy and the orbita had been invaded, we performed craniofacial resection instead of endoscopic resection (Fig. C2A). We drilled into the right side of the crista galli, midline of the cribriform plate, and perpendicular plate of the ethmoid bone via craniotomy. As a result, we accessed the nasal cavity directly (Fig. 2B). To preserve the nasal septum, we detached the remaining right septal mucosa through the transfacial approach (Fig. 2C). Because of the high risk of cerebrospinal fluid leakage as a result of previous irradiation, we performed vascularized free flap reconstruction of the skull base instead of pericranial flap.Postoperative computed tomography revealed no evidence of tumor (Fig. 1C, D). The patient's sense of smell returned after 1 postoperative day, and she was discharged on the postoperative day 14.The link to the video can be found at: https://youtu.be/XzPABYwzkjs.

Musculoskeletal oncology: patient triage and management during the COVID-19 pandemic

Sarcoma treatment during the covid-19 pandemic is a new challenge. This patient population is often immunocom­promised and potentially more susceptible to viral complications. Government guidelines highlight the need to minimize patient exposure to unnecessary hospital visits. However, those guidelines lack practical recommendations on ways to manage triage and diagnosis expressly for new cancer patients. Furthermore, there are no reports on the efficiency of the guidelines. One of the main issues in treating musculoskeletal tumours is the complexity and variability of presentation. We offer a triage model, used in a quaternary-referral musculoskeletal oncology centre, that allows us to maintain an open pathway for referral of new patients while minimizing exposure risks. A multidisciplinary approach and analysis of existing investigations allow for a pre-clinic evaluation. The model identifies 3 groups of patients: Patients with suspected high-grade malignancy, or benign cases with aggressive features, both in need of further evaluation in the clinic and prompt treatment Patients with low-grade malignancy, and benign cases whose treatment is not urgent, that are managed, during the pandemic by telemedicine, with reassurance and information about their illness Patients who can be managed by their local medical professionals In comparison to a pre-pandemic period, that approach resulted in a higher ratio of malignant-to-benign con­ditions for new patients seen in the clinic (3:4 vs. 1:3 respectively), thus using available resources more efficiently and prioritizing patients with suspected high-grade malignancy. We believe that this triage system could be applied in other surgical oncology fields during a pandemic.

Asymptomatic Unilateral Primary Adrenal Lymphoma Mimicking a NonFunctioning Adenoma in Adult Male with a Good Outcome

Primary adrenal lymphoma (PAL) is a type of extranodal lymphoma that is rarely encountered in clinical settings. It is regarded as a high-grade malignancy with a very poor prognosis. Most PALs are bilateral and hardly unilateral. Nevertheless, a rise in the number of cases of bilateral and unilateral PALs has been noted in the past few years, with a wide range of presenting symptoms from fatigue to adrenal insufficiency. Many were found incidentally via radiology imaging or on postmortem examinations. This paper aims to report an unusual case of a unilateral primary adrenal non-Hodgkin’s lymphoma in a previously healthy 53-yearold Bahraini male, who presented with vague yet persistent right flank pain of a few months’ duration. Imaging modalities revealed a large right adrenal mass measuring 11x10x9 cm which was initially thought to be an adenoma on CT scan, but post laparoscopic surgical excision was diagnosed histologically as Diffuse Large B Cell Lymphoma (DLBCL). Cycles of Rituximab-CHOP chemotherapy was given, and on regular follow up for two and a half years, the patient remains disease-free with no signs of recurrence.

Importance of Early Diagnosis of Gastrointestinal Stromal Tumors of the Stomach: Our 5-Year, Single-Center Experience

Objective: The aim of this study was to investigate clinicopathologic characteristics, diagnosis, treatment, and prognosis of patients who underwent surgery with a diagnosis of a gastrointestinal stromal tumor (GIST) of the stomach. Methods: In this single center study, the data of a total of 42 patients diagnosed with a GIST of the stomach in our center between 2010 and 2015 were retrospectively analyzed. The mean age was 60.6 years, with a male-to-female ratio of 1.47/1. Patients presented with various complaints (21 patients had stomach pain,14 patients were asymptomatic, 3 patients had hemorrhage, 2 patients had heartburn and acidity,1 patient had intumescence,1 patient had nausea and vomiting), and all patients underwent surgery. The initial diagnosis was made with endoscopy in 19 patients, with computed tomography in 21 patients, and with magnetic resonance imaging in 2 patients. Local excision or wedge resection was performed in 27 patients, whereas subtotal gastrectomy was performed in 15 patients. Results: The mean tumor diameter was 5.66 cm. Pathologically, 25 patients had very low, 5 patients had low, 6 patients had moderate, and 6 patients had high-grade malignancy. The patients with moderate- to high-grade malignancy received imatinib. Liver metastasis occurred in 2 patients. The mean follow-up was 33 months. The mean disease-free survival time was 31.72 months, and the mean disease-specific (n = 2) survival time was 31.25 months. All patients including metastatic ones were still alive. Conclusions: Our study results show that frequent use of imaging studies and increased use of endoscopic scans for various reasons may increase the rate of incidentally detected gastric stromal tumors. High rates of disease-free survival can be achieved in GIST patients who are in the low-risk group due to the early diagnosis.

Primary pleomorphic liposarcoma of fallopian tube with recurrence: a case report and review of the literature

BackgroundLiposarcoma, which develops in adipose tissue, is one of the most common soft tissue sarcomas. It appears mostly in the lower limbs, particularly in the thigh and limb girdles, followed by the upper extremities, thoracoabdominal wall, and the internal trunk and retroperitoneum. Pleomorphic liposarcoma (PLS), a rare subtype of liposarcoma is considered a highgrade malignancy.Case presentationWe present a case of primary PLS in the left fallopian tube of a 47-year-old female. Ten months previously, she was diagnosed with PLS of the left fallopian tube in another hospital and had a wide excision of left fallopian tube mass, including total abdominal hysterectomy and bilateral salpingo-oophorectomy for left fallopian tube PLS. Presently, she has developed a recurrence and metastasis of PLS in the pelvic and abdominal cavities.ConclusionThis may be the first case of primary fallopian tube PLS, wherein the prognosis of this patient was poor due to the high-grade malignancy of PLS.

Dedicated breast PET to improve clinical diagnosis of breast cancer: Initial evaluation.

e12097 Background: Unlike whole body (WB) 18F-fluorodeoxyglucose (FDG) PET, dedicated breast PET (DBPET) enables detailed high-resolution images within the breast and quantitative assessment using SUV. We aimed to determine whether DBPET can detect ductal carcinoma in situ (DCIS) and predict the malignancy and neoplastic progress of invasive breast cancer. Methods: We investigated 155 consecutive patients with breast cancer who underwent concurrent DB– and WB– PET (Elmammo®) between January and November 2016. All DB– and WB– PET images were quantified based on standard uptake values (SUV). We also assessed 12 patients with ring-like uptake (RU) without central FDG accumulation in invasive breast cancers on DBPET images. Results: The SUV (means ± SD) determined from DB– and WB– PET images of breast cancer were 8.2 ± 7.7 and 3.1 ± 3.3 respectively. We detected DCIS in DB- and WB– PET images from 22 (84.7%) and 15 (57.6%) of 26 among the 155 patients, respectively (p = 0.003). The SUV on WB– PET images was significantly higher for NG3 than for NG1-2 (p = 0.004), and for high (≥ 20%) than low ( < 20%) Ki67 (p = 0.003), and significantly lower for Luminal A than Luminal B, HER2 and triple-negative cancer (p < 0.05 for all three). The SUV for NG (p = 0.002), Ki67 (p = 0.001) and the luminal (p < 0.05) type also significantly differed in DBPET images. RU without central FDG accumulation was evident in DbPET images of 12 patients, all of whom had Ki67 ≥ 20 (p = 0.0001), two had NG1-2 and 10 had NG3 (p = 0.005), one had clinical T1, 11 had T2 (p = 0.0004), five had clinical N0 and seven had N1 (p = 0.03). RU was associated with high-grade malignancy and neoplastic progress. Conclusions: The detection rate of DBPET was excellent for DCIS. The RU on DBPET offers predictive value for high-grade malignancy and the neoplastic progress of invasive breast cancer. Thus, DBPET might help to determine therapeutic strategies.