Secondary Trigeminal Neuralgia: a Case Report and Literature Review of Red Flags

Introduction: Trigeminal neuralgia (TN) is characterized by recurrent paroxysmal brief episodes of electric shock-like pain along the trigeminal nerve distribution. Based on the underlying cause, current classification systems have classified TN into idiopathic, classical, and secondary TN. This manuscript presents a case report of a patient seen in the clinic with features of trigeminal neuralgia secondary to an intracranial lesion. Case description: A 39-year-old female presented to the clinic with a 15-month history of severe, intermittent, short-lasting episodes of pain affecting the left lower teeth, jaw, nose, and temporal region. The patient reported familiar shock-like pain during the physical examination when the skin of the left ala of the nose was lightly touched. The rest of the clinical examination was non-significant. The magnetic resonance imaging of the brain showed an approximately 20 mm wide lesion at the level of the cerebellopontine angle. After subsequent tests, the lesion was diagnosed as meningioma, and the patient was treated with stereotactic radiation therapy. Practical Implications: In up to 10 % of TN cases, the underlying cause can be due to a brain tumor. Although persistent pain, sensory or motor nerve dysfunction, gait disturbances, and other neurological signs may concurrently exist, raising a red flag for intracranial pathology, patients often present with pain alone as the heralding symptom of a brain tumor. Due to this, it is imperative that all patients suspected of having TN undergo an MRI of the brain as part of the diagnostic work-up.

Infantile T-cell Acute Lymphoblastic Leukemia: a Case Report

Introduction: Acute lymphoblastic leukemia (ALL) is the most common malignancy in children, with a male predominance. Pediatric acute lymphoblastic leukemia is usually of B-cell lineage; T-cell leukemia is uncommon and extremely rare under one year of age. Mixed-lineage leukemia gene rearrangement is the best-known hallmark of infantile leukemia and is a poor prognostic indicator. While multi-agent high dose chemotherapy remains the first line of treatment for pediatric T-ALL, there are numerous side effects of these regimens, and most patients undergo relapse. Due to the rarity of the disease, treatment protocols for infantile T-ALL have not been established to date. Clinical description: We present a case of a 7-month-old Pakistani male that presented with fever and cough and was subsequently diagnosed with T cell acute lymphoblastic leukemia. T-ALL was diagnosed on flow cytometry. Due to poor prognosis, the patient was assigned palliative care. Practical implications: Management of infantile leukemia has yet to be studied in-depth. With a lack of clear treatment guidelines, the approach towards these patients remains challenging. Further research and clinical trials in this area of study are paramount to improving clinical outcomes for these young patients.

Unusual Presentation of Treated Colon Cancer with Extramural Venous Invasion: a Case Report

Introduction: Colon cancer is one of the leading malignancies globally and continues to be one of the most typical causes of cancer-related mortality. The clinical outcome of the disease depends on the primary tumor stage, regional nodal involvement, and distant disease dissemination. It often presents with hematogenous spread to the liver at the time of diagnosis. Another factor for increased mortality is the presence of extramural venous invasion (EMVI). This is exceedingly important as it has significant prognostic significance and helps predict survival. Case Description: A middle-aged female with a recent history of cesarean delivery presented with abdominal pain and occasional constipation, which led to a series of investigations. Initial Computed Tomography (CT) scan showed proximal to mid transverse colonic tumoral thickening with locoregional lymphadenopathy and solitary distant metastasis in the left hepatic lobe. This was followed by extended right hemicolectomy and hepatic metastasectomy. The patient remained on follow-up and later presented with thrombus formation in the splenoportal circulation. Initially, this was considered a bland thrombus, and the patient was advised a close follow-up. However, the patient was lost to follow and later presented with extensive thrombosis of the portal and splenic veins. Practical Implications: Confident differentiation of the bland versus malignant thrombosis is crucial to ascertain disease stage and appropriate management. Invasive tissue sampling gives a confident diagnosis of benign versus malignant thrombus. However, using a noninvasive imaging modality, we can still distinguish between the two with reasonable certainty.

Presentation of Acute Lymphoblastic Lymphoma and Colorectal Carcinoma in the Context of Constitutional Mismatch Repair Deficiency Syndrome (CMMRD): a Case Report with Literature Review

Introduction: Constitutional mismatch repair deficiency (CMMRD) is a rare autosomal recessive disease-carrying an increased risk of cancers (pediatric tumors of central nervous system, haemato-lymphoid malignancies along with gastrointestinal (GI) cancer(s), which are usually seen in the second and third decade) leading to syndromic presentation. Causal mutations are detected in DNA mismatch repair (MMR) genes, including MLH1, PMS2, MSH2, and MSH6 that are also known for their established role in Lynch syndrome. We describe a case of CMMRD with an earlier (first decade of life) presentation of mediastinal acute lymphoblastic lymphoma and colorectal malignancy. Case Presentation: A five-year-old boy presented with respiratory complaints, bilateral cervical lymphadenopathy, multiple café au lait macules (CALMs) on the lower back, history of parental consanguinity with the death of three sisters due to brain tumor within 6 months of diagnosis. Computerized tomographic (CT) scan chest revealed a huge mediastinal mass. The patient underwent a trucut-biopsy of the mass. The results were significant for a pre T-cell acute lymphoblastic lymphoma. Suspicion of CMMRD was raised based on a combination of factors described above. A panel of mismatch repair (MMR) proteins was applied on the biopsy tissue that revealed loss of nuclear expression of MLH1 and PMS2 immunostaining in tumor cells with positive external controls. While on maintenance therapy for lymphoma, about a year later, the patient developed sub-acute intestinal obstruction due to a stenosing polypoidal circumferential tumor in the mid-sigmoid colon found on flexible sigmoidoscopy that was followed by endoscopic biopsies and insertion of a fully-covered self-expanding metallic adult biliary stent with a diameter of 10 mm and length of 6 cm leading to immediate relief of obstruction. Biopsies revealed adenocarcinoma with neuroendocrine differentiation. Metastatic tumor deposits were seen in the omentum, anterior abdominal wall, and the left peritoneal wall. Practical Implications: Earlier (first decade) presentation of gastrointestinal malignancy warrants that an earlier screening through radiological scans for any possible tumors and MMR protein expression analysis (loss in tumor plus normal non-tumor cells) are essential in patients having CALMs and family history of pediatric tumors.

Primary Prostatic Diffuse Large B-Cell Lymphoma: a Case Report and Literature Review

Introduction: Primary lymphomas of the prostate are globally rare representing less than 0.1% of all prostatic neoplasms. In this paper we present a case of an early stage diffuse large B-cell lymphoma (DLBCL) of the prostate managed with six cycles of rituximab-based chemotherapy, and review the related literature. Case description: A 32-year-old man presented to our clinic with complaints of difficult urination and perineal pain. An enlarged, hard and nodular prostate was palpable on digital rectal examination. Needle biopsy of the prostate was performed, which revealed diffuse large B-cell non-Hodgkin's lymphoma by immunohistochemical studies. CT scan showed large pelvic mass arising from prostate encasing ureters with bilateral hydronephroureter. No abnormal finding was seen on abdominal CT and bone marrow histology. Therefore, the disease was classified into the clinical stage IAXE according to Ann Arbor's criteria. The patient achieved complete response (CR) to six cycles of rituximab based combination chemotherapy, R-CHOP with CNS prophylaxis. He remained disease free, until now, 36 months after the end of chemotherapy. Practical Implications: According to the literature, the treatment and prognosis of primary lymphoma of the prostate is the same as that of other nodal lymphomas. Rituximab-based regimen should be considered in the management of prostatic diffuse large B-cell lymphoma.

A Case of Esophageal Squamous Papilloma, an Unusual Cause of Dysphagia and Hematemesis in a Patient with Concurrent Malignancies

Introduction: The oesophageal squamous papilloma (ESP) is a rare cause of dysphagia and hematemesis. The malignant potential of this lesion is uncertain; however, the malignant transformation and concurrent malignancies have been reported in the literature. Case description: We report a case of oesophageal squamous papilloma in a 43-year-old female who had a background diagnosis of metastatic breast cancer and liposarcoma of the left knee. She presented with dysphagia. Upper gastrointestinal (GI) endoscopy showed a polypoid growth, and its biopsy confirmed the diagnosis. Meanwhile, she presented again with hematemesis. A repeat endoscopy showed that the previously seen lesion had likely broken off, leaving behind a residual stalk. This was snared and removed. The patient remained asymptomatic, and a follow-up upper GI endoscopy at six months did not show any recurrence. Practical implications: To our knowledge, this is the first case of ESP in a patient with two concurrent malignancies. Moreover, the diagnosis of ESP should also be considered when presenting with dysphagia or hematemesis.

Extraskeletal Mesenchymal Chondrosarcoma, a Rare Entity with Unusual Metastases: A Case Report

Introduction: Extraskeletal mesenchymal chondrosarcoma (ESMC) is rare, aggressive, and high grade malignant tumors originating from soft tissues. It carries a poor prognosis with a tendency for local recurrence and distant metastasis, necessitating long-term follow-up. The most common sites for metastasis are the lungs, bones, and lymph nodes. Meanwhile, pancreatic metastases are extremely rare. Case Description: A 35-year-old female presented with a history of wide local excision for the left upper limb mass; histopathology showed ESMC. She was on surveillance with a computed tomography scan of the thorax and magnetic resonance imaging of the left upper limb at 3-months intervals until she developed vertebral and pancreatic lesions after 6 months post-surgery. No pulmonary metastases were noted. Considering the unusual site for metastasis and to exclude the possibility of any second malignancy, bone biopsy, and endoscopic ultrasound-guided fine-needle aspiration was performed that confirmed metastases. Later she developed osseous metastases in the pelvis and femora. Practical Implication: Pancreatic metastasis from ESMC is extremely rare. In case of new visceral or osseous lesions in a patient with a past medical history of ESMC, the possibility of metastatic disease should be considered. A biopsy can be performed to confirm the diagnosis.

Improving Compliance of Physicians in Reporting and Documenting Critical Alerts in a Cancer Hospital

Introduction: A critical result of an investigation is considered a representation of a pathophysiological state deemed to be high-risk or life-threatening for the patient. Therefore, such results should be addressed in an appropriate and timely manner. Unfortunately, routine closed-chart audits suggested that the compliance of physicians in documenting critical alerts in patient notes was poor. This prompted the hospital to conduct a continuous quality improvement (CQI) project to improve the physicians' compliance. Materials and Methods: A cause-and-effect analysis was conducted using a fishbone diagram to identify the reasons for poor compliance. Based on the analysis, several modifications were made, including, but not limited to, hospital-wide educational sessions on the standard operating procedures of receiving and documenting critical alerts for the physicians, daily audit of critical alerts to review the appropriateness of documentation, and introduction of a new module in the hospital electronic medical record to acknowledge and document receiving critical alerts. Results: Before implementing the strategies to improve physicians' documentation compliance, the average compliance rate was 57 % in April 2020, and the median compliance rate was 52 % (January 2020 - April 2020). However, afterwards, within a couple of months of implementing changes, the average compliance rate increased to 88 %. This improvement was sustained for the next eight months (median of 89 %). Conclusion: This study found that CQI approach can be used to improve the compliance of the physicians for appropriately and timely documenting critical alerts, in this case, by continued education and training process and incorporating changes into the electronic hospital information system.

Extramedullary Plasmacytomas of the Oral Cavity: A Case Report and Review of the Literature

Introduction: Multiple myeloma is a hematologic malignancy characterized by the proliferation of plasma cells and typically presents with lesions in bone, known as plasmacytomas. Through hematogenous spread, extramedullary plasmacytomas can develop in soft tissue in any location of the body. This case report describes a patient with multiple myeloma who presented with an extramedullary plasmacytoma on his maxillary gingiva and provides an updated review on the classification and characterization of extramedullary plasmacytomas of the oral cavity. Case description: A 53-year-old male with a known diagnosis of multiple myeloma was referred to our clinic for evaluation of a gingival nodule, which was tender to palpation and had been present for a month. Clinical examination revealed a 1.5 cm violaceous, red nodule of the maxillary buccal attached gingiva, which did not blanch on palpation. He had a similar 1 cm, smooth, red nodule of his cutaneous skin on his left arm. Radiographic examination with within normal limits without evidence of dental or bony pathology. An incisional biopsy revealed the diagnosis of plasmacytoma, indicating relapse and progression of the patient’s multiple myeloma.Practical implications: Multiple myeloma can present in the oral cavity either as intra-bony plasmacytomas, paraskeletal plasmacytomas, or extramedullary plasmacytomas in the soft tissue. Extramedullary disease representative of hematogenous spread is concerning for high-risk disease with a poor risk prognosis.

Efficacy of Chemotherapy for Locally Advanced and Metastatic Pancreatic Cancer: A real life experience and outcome from a tertiary referral centre.

Introduction: To report response rate, progression-free survival and overall survival in patients with advanced pancreatic cancer treated with different available chemotherapeutic regimens over ten years. Materials and Methods: This is a retrospective observational study. All patients with locally advanced and metastatic pancreatic cancer at Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan, from January 2008 to December 2017 were studied. Data were collected from the hospital information system. The characteristics and outcomes of all the patients were analyzed. Progression-free survival and overall survival were also estimated. Kaplan Meier curves and Log-rank test were applied, and SPSS version 20 was used for data analysis. Results: Eighty-seven (87) subjects with a median age of 56 years (range 21-76) were included. Sixty-two (71%) subjects were male. The most common tumor location was the head of the pancreas in 46(53%) of all the subjects. Sixty-three (72%) subjects had elevated CA-19.9 values. About 47(54%) subjects had locally advanced pancreatic cancer (LAPC), and 40(46%) subjects had metastatic pancreatic cancer (MPC). Chemotherapy regimens used were FOLFIRINOX in 23(26%), gemcitabine-based 66(65%) and capecitabine-based in 8 (9%) of the subjects. One (1%) subject had a complete response (CR), 12(14%) had a partial response (PR), 10 (11%) had stable disease, and 59(68%) of the subjects had progressive disease (PD). The objective response rate (ORR) was 15%, and the disease control rate (DCR) was 26%. In MPC, the ORR was 10%, DCR was 18%, and tumor progression was seen in 72% of the patients, while in LAPC, the ORR was 19.1, DCR 34% and tumor progression was documented in 64% of the patients, respectively. The FOLFIRNOX chemotherapy regimen had better ORR, DCR and lesser number of progressions as compared to Gemcitabine and Capecitabine based chemotherapy regimens. The Median PFS of the whole group was 32-weeks, and the median OS was 54-weeks. The PFS was significantly higher for LAPC (39 weeks) as compared to the MPC group (25 weeks) (p=0.028). There was no statistically significant difference between the OS of these 2 groups (p=0.451). In addition, PFS was significantly higher with FOLFIRINOX chemotherapy as compared to the other chemotherapy regimens. Regarding OS, there was no statistically significant difference among all chemotherapy regimen groups (p=0.267). Conclusion: Based on our results, FOLFIRINOX remained the most effective chemotherapy regimen despite the dose modifications and toxicities in all groups, indicating that modified FOLFIRINOX could be considered as a first-line regimen in south East Asian population.