Journal of Cancer and Allied Specialties
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Clinical Characteristics and Treatment Outcome of Pediatric Rhabdomyosarcoma; Retrospective Review
Introduction: Rhabdomyosarcoma is the most common soft tissue sarcoma in children. This paper aimed to assess the stage, site, and treatment outcome among rhabdomyosarcoma (RMS) patients. Materials and Methods: A retrospective chart review was completed from January 2011 to December 2017 of patients that presented to the Department of Pediatric Oncology, Shaukat Khanum Memorial Cancer Hospital, Lahore, Pakistan, for the management of RMS. Data collection included clinical characteristics, staging, grouping, risk stratification, treatment plan, radiotherapy doses, and treatment outcome. Results: Among 24 subjects, there were a total of 13 (54.2%) males and 11 (45.8%) females. The median age at the time of diagnosis was 2.5 years (range: 0.75 - 17 years). The majority of the subjects (91.7%) were less than 10 years of age. The median follow-up time was 0.6 years. According to the Children's Oncology Group Classification, 4 (16.7%) subjects were classified as low risk, 14 (58.3%) subjects were rated as intermediate risk, and 6 (0.25%) subjects were stratified as high risk. The most common primary tumor site were genitourinary (62.5%) and abdomen/retroperitoneal (20.8%) regions. At the time of analysis, nine (37.5%) subjects had died because of the disease, twelve (50%) were alive with no evidence of disease, and one subject had a recurrence of disease and was alive. One subject had abandoned the therapy, and another was lost to follow-up. Conclusion: Patients with Rhabdomyosarcoma presented at the late stages of the disease, and it most frequently affected genitourinary and abdomen or retroperitoneal areas. Overall, Rhabdomyosarcoma was found to have a poor outcome to therapy
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
Editorial: COVID-19 pandemic, the second wave, and provision of cancer services.
SARS-CoV 2 (COVID-19) pandemic continues to have a debilitating effect on health worldwide. This editorial provides an overview of the provision of cancer services at Shaukat Khanum Memorial Cancer Hospital & Research Centre, Lahore, and Peshawar. 
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
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
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
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
Myasthenia Gravis Mimicking Third Cranial Nerve Palsy: a Case Report
Background: The most common pituitary adenoma presentation is a visual field defect and inappropriate pituitary hormone secretion. The compression of the optic chiasma causes visual impairment. Large pituitary adenomas can rarely cause diplopia and ptosis secondary to adenoma's lateral extension into the cavernous sinus. Myasthenia gravis (MG) is an autoimmune disorder involving neuromuscular junctions. It is characterized by skeletal muscle fatigability, commonly involving extraocular muscles, face, and limbs. It is estimated that 75% of MG patients present with ptosis and diplopia. The association of MG with pituitary adenoma is very rare. Case Description: A 30-year-old lady presented with headache, diplopia, and ptosis of the left eye for two months. She was diagnosed with acromegaly secondary to pituitary adenoma. Ptosis is a rare presenting feature in pituitary adenoma. Her case was discussed in a multidisciplinary meeting, and the consensus was that her ptosis is likely secondary to pituitary adenoma, which was involving the left cavernous sinus. She underwent transsphenoidal resection of pituitary macro adenoma. Three weeks post-surgery, she developed bilateral ptosis, dysarthria, dysphonia, which was diagnosed as myasthenia gravis. Clinical implications: Ptosis is a rare manifestation of pituitary adenoma. Nonetheless, pituitary tumor patients presenting with ptosis should be evaluated for the neuromuscular disorder. A high index of suspicion is required for early diagnosis and prompt treatment of myasthenia gravis
Ectopic Acromegaly Secondary to Bronchial Tumor; a Case Report of Rare Occurrence.
Introduction: Acromegaly is caused due to the unregulated and sustained overproduction of growth hormone (GH). The majority of the cases are caused by autonomous secretion of GH from anterior pituitary tumors. Nonetheless, in less than 1 % of the cases, the cause of autonomous secretion is secondary to ectopic growth hormone-releasing hormone (GHRH) production. Bronchial carcinoids are the most common cause of ectopic GHRH production. Case description: A 32-year-old female presented to the clinic with a history of cough, hemoptysis, and undocumented weight loss for four years. Initial workup showed a large right main stem endobronchial mass. Transbronchial biopsy of the mass revealed a grade I neuroendocrine tumor (NET). During NET workup, a large sellar mass was incidentally found on cross-sectional imaging. The hormonal profile revealed markedly elevated insulin-like growth factor -1 (IGF-1) and mildly raised prolactin. The MRI Brain study revealed pituitary macroadenoma measuring 2 cm x 1.2 cm x 1.5 cm. The patient underwent bronchial carcinoid tumor resection, which led to normalization of serum IGF-1 and growth hormone response to an oral glucose tolerance test. Subsequent MRI brain revealed complete resolution of previously noted sellar mass. Practical implications: This case highlights the importance of differentiating acromegaly secondary to pituitary adenoma and ectopic acromegaly. This case emphasizes the importance of keeping rare entities in the differential while assessing patients with pituitary macroadenoma
Expression of B-cell Lymphoma 2 (BCL-2) in Breast Cancer
Introduction: Immunohistochemical expression of B-cell lymphoma 2 (BCL-2) is seen variably in invasive ductal carcinoma. This study was conducted to determine the frequency of BCL-2 expression in different histologic grades of invasive ductal carcinoma. Materials and Methods: A cross-sectional study was conducted in the Department of Pathology at Shaukat Khanum Memorial Cancer Hospital and Research Centre, Pakistan, on subjects with invasive ductal carcinoma of various histologic grades. Immunohistochemistry was done using the BCL-2 antibody in all cases. The frequency of BCL-2 positive cases in different histologic grades was noted. Post-stratification, the chi-square test was applied. A p-value ≤ 0.05 was considered significant. Results: All 52 subjects were females (100%) with a mean age of 47.58 ± 1.43 years. BCL-2 expression was observed in 28 (53.85 %) subjects with breast cancer. Out of 33 participants with grade III, 13 (39.39 %) participants were positive for BCL-2 expression. Among 18 subjects with grade II, 14 (77.78 %) subjects were positive for BCL-2 expression. Reduced frequency of BCL-2 expression was observed with increasing histologic grade (i.e., more in low-grade tumors and less in grade III), but the difference was statistically not significant. Conclusion: A differential expression of BCL-2 was observed across different grades of invasive ductal carcinoma. However, the difference was not statistically significant