Nagesh T Sirsath

Paranasal Sinus Neuroendocrine Carcinoma: A Case Report and Review of the Literature

Neuroendocrine neoplasms are defined as epithelial neoplasms with predominant neuroendocrine differentiation. They can arise in almost every organ of the body although they are most commonly found in the gastrointestinal tract and respiratory system. Nasal cavity and paranasal sinuses are a rare site for neuroendocrine carcinoma. In contrast to the other regions, neuroendocrine tumours of the sinuses have been reported to be recurrent and locally destructive. Very few cases of paranasal sinus neuroendocrine carcinoma have been reported till date. Difficulty in pathologic diagnosis and rarity of this malignancy have hindered the progress in understanding the clinical course and improving outcomes. We herein report a case of poorly differentiated neuroendocrine tumour of ethmoid and sphenoid sinus with invasion of orbit and intracranial extension. The patient had complete response at the end of chemoradiation and he was disease-free for 9 months duration after which he developed bone metastasis without regional recurrence.

An unusual case of pulmonary granulocytic sarcoma treated with combined chemotherapy and radiation.

We report an unusual case of a 6-year-old male child who presented with fever and a cough of one month’s duration. A bone marrow aspiration and cytogenetics were suggestive of acute myeloid leukaemia with t(8;21)(q22;q22). A chest x-ray and computed tomography of the thorax showed a soft tissue lesion in the right lung. The fine needle aspiration cytology (FNAC) of this lesion was suggestive of pulmonary granulocytic sarcoma. The patient was successfully treated with induction chemotherapy (cytosine arabinoside + daunomycin), followed by consolidation with high-dose cytosine arabinoside. In view of the persistent lesion in the right lung, the patient was given external beam radiotherapy (EBRT), which resulted in near total resolution of the lung granulocytic sarcoma. We report this case in view of its rarity and clinical importance, and to highlight the treatment options in this scenario.

Locally advanced oral cavity squamous cell carcinoma: Barriers related to effective treatment

Background: Oral cavity cancer is a significant health problem in India. Majority of patients present with locally advanced disease requiring multimodality treatment. Compliance to recommended treatment is an important factor affecting outcome. Aims: The aim was to evaluate the outcome of locally advanced oral cavity cancer patients with regards to treatment adherence and to assess reasons of noncompliance. Materials and Methods: This was a prospective observational study. We included patients referred to Department of Medical Oncology for induction chemotherapy in view of locally advanced oral cavity cancer. Results: Only 15 (26%) patients completed planned treatment schedule. Their 1 year overall survival was 93%. The remaining 43 patients who received inadequate treatment had a dismal 21% 1 year overall survival. Illiteracy, poverty, long waiting list for surgery, prolonged delay for health scheme treatment plan approval and dissatisfaction with attitude of hospital staffs are major barriers related to effective treatment of these patients. Conclusions: A detailed discussion with patient and their relatives regarding recommended treatment, proper implementation of health schemes, increasing trained manpower to avoid long waiting list for surgery, provision of additional financial support for family member accompanying the patient and a sympathetic approach toward patients are needed to help these patients overcome the battle.

Plasmablastic lymphoma: Does prognosis differ with HIV status and site of disease?

Background: Apart from its common occurrence in the oral cavity in HIV-positive patients, plasmablastic lymphoma (PBL) has also been described at extraoral sites and among immunocompetent individuals. There is sparse data quoting prognostication of PBL depending on the site of occurrence and HIV status of patients. Aims: The present study was carried out at a tertiary oncology center to address the issue whether PBLs occurring at oral and extraoral sites differ prognostically and whether HIV status of patient has any impact on prognosis. Materials and Methods: This was a retrospective observational study conducted at our center on consecutive patients diagnosed with PBL, from January 2008 to December 2012. Results: We had four patients with oral PBL; three male and one female. Sites of involvement were oral tongue and buccal mucosa. Two patients died within 6 months of diagnosis due to disease progression while on treatment. One patient was lost to follow-up after achieving complete remission (CR) after chemotherapy. Only one patient completed the prescribed schedule of chemotherapy and radiotherapy and is in CR with 33 months follow-up. There were four extraoral PBL patients; three female and one male. Extraoral sites were ileocaecal region, ovary, clavicle and rectum. Three patients died within 6 months due to progressive disease during treatment. Only one patient has completed chemotherapy and is in CR with18 months follow-up. Among all these eight oral and extraoral PBL patients, four were HIV positive. Two of them are in CR after treatment (18 months and 33 months follow-up). One patient died during treatment and one patient was lost to follow-up after being in CR. Unfortunately, none of the other four HIV-negative patients could survive for more than 6 months after diagnosis. Conclusion: Both oral and extraoral PBLs have aggressive clinical course and an overall unfavorable outcome. Prognosis of HIV-associated PBL seems to be better with addition of highly active antiretroviral therapy to chemotherapy. Further large sample studies are needed to confirm these results.

Human Immunodeficiency Virus – Associated Lymphomas: A Neglected Domain

Background: Human immunodeficiency virus (HIV) associated lymphoma is an important public health concern; however, the epidemiological data available from India is sparse. Aims: The present study was carried out at a tertiary cancer care center in South India to analyze the scenario of HIV-associated lymphoma. Materials and Methods: This was a retrospective observational study conducted at our center, on consecutive patients diagnosed with HIV-associated lymphoma, from January 2008 to December 2012. Results: A total of 44 patients were diagnosed with HIV-associated lymphoma, of which 18 opted for treatment. There were 11 males and 7 females in the study population. Median interval from the diagnosis of HIV infection to diagnosis of lymphoma was 18 months. Median CD4 count at the time of lymphoma diagnosis was 218/mm3. Five patients had Hodgkins lymphoma, and the rest had non-Hodgkins lymphoma. Five out of 18 (28%) patients in the present study expired during treatment. Ten (55.5%) patients are alive and lymphoma free, with a median follow up of 18 months. Conclusions: More than half of our treated patients are lymphoma free with a median follow up of 18 months; hence treatment of patients with HIV-associated lymphoma should be encouraged.

A complex variant t(3;15) (q26;q13) representing cryptic/masked acute promyelocytic leukaemia with a novel breakpoint of chromosome 15-a case report.

Acute promyelocytic leukaemia (APML) is a biologically and clinically distinct variant of AML, currently classified as acute myeloid leukaemia with recurrent cytogenetic anomalies t(15;17) (q22;q21), promyelocytic leukaemia-retinoic acid receptor alpha, diagnosis regardless of blast count in the World Health Organization classification system. It is one of the curable malignancies, has a unique clinical presentation, often with disseminated intravascular coagulation, and has a targeted therapy for its treatment in the form of all trans retinoic acid (ATRA) and arsenic trioxide (ATO). Here, we report a complex type of variant APML t(3;15) (q26;q13), the need for conventional karyotyping for diagnosing such rare variants, and its response to ATRA and ATO.

Carotid blowout syndrome: An oncological emergency less discussed

South Asian Journal of Cancer ♦ Volume 6 ♦ Issue 2 ♦ April-June 2017 85 the carotid artery is exposed to the oral cavity or external environment through skin breakdown or by direct tumor invasion; “impending,” in which sentinel bleeds have occurred but have been controlled by conservative management; or “acute,” where there is active bleeding.[5] The gold standard for diagnosing CBS is digital subtraction angiography. In patients presenting with threatened and impending CBS, a CT angiogram of the head and neck is reasonable to evaluate the carotid circulation up to the circle of Willis.[6] Following diagnosis, immediate treatment is crucial and focuses on aggressive critical care with securing the airway and management of hemorrhagic shock.[7] Traditional surgical ligation had been the only choice in the past with a displeasingly high rate of neurological morbidity and mortality due to altered anatomy as a result of prior surgery or radiation. Hence, CBSs main treatment has shifted to endovascular techniques: either embolization or stenting of parent artery.[4] Lesley et al. describe a 15%–20% rate of acute or delayed cerebral ischemia following occlusion, which can be predicted by a balloon occlusion test. This test may however be bypassed in acute cases.[8] Endovascular stents are considered in patients who are at high risk for cerebral ischemia such as those who have an incomplete circle of Willis or occluded contralateral common carotid artery. With increased availability and smaller delivery systems, covered stents are an attractive alternative for CBS.[9] However, long-term results have been less encouraging, with high rates of technical complications including cerebral thromboembolism by the inadequate antiplatelet medication, septic thrombosis of the stent graft, and delayed stenosis/ occlusion of the carotid artery by the strong radial force of the stent graft.[10,11] Patients with acute CBS were associated with a higher rebleeding rate than those with impending and threatened CBS. Rebleeding occurs due to reconstitution of collateral vessels or recanalization of the thrombosed carotid artery. Cross occlusion is preferred in such cases to enhance durable hemostasis. For all patients, clinical severity is the significant factor affecting the hemostatic outcome of endovascular management.[12] A sequential approach of treating impending CBS with induction chemotherapy, embolization, and radiation therapy has also been successfully employed.[13] Carotid blowout syndrome: An oncological emergency less discussed

Diffuse large B‑cell lymphoma of the thyroid: Seven cases with review of the literature from India

Background: Diffuse large B-cell lymphoma (DLBCL) is the most frequent subtype of nonHodgkin′s lymphoma globally with frequent extra nodal involvement. There is sporadic data available on thyroid DLBCL in Indian population and hence we undertook this retrospective observational study at Kidwai Memorial Institute of Oncology, a tertiary care oncology center in India to analyze the clinico biological features of this disease. Methodology: A total of 7 consecutive cases diagnosed as DLBCL by appropriate lymph node biopsy with evidence of thyroid involvement on fine-needle aspiration cytology of thyroid or thyroid histopathology (following thyroidectomy) were confirmed by immunohistochemistry (WHO classification) between January 2008 and September 2013 were included in this study. Results: A total of 7 patients were included in the study. Median age was 65-years (range: 50-72 years), and all were females. Six out of seven were in stage IIAE and one was in stage IVAE. The distribution according to the international prognostic index was as follows: 3 were in low risk, 1 in low-intermediate, 2 in high-intermediate and 1 in high risk group. Regarding treatment, 5 received CHOP, 1 received COP and 1 received no treatment. 5 patients received radiotherapy. 2 patients underwent subtotal thyroidectomy. Of the 6 patients who received chemotherapy ΁ radiotherapy, 4 had complete response 1 was lost to follow-up at 3 months and 1 alive with no disease at 32 months), 2 had partial response. The median survival was 15 months (3-32 months). Conclusions: Extranodal thyroid DLBCL presents common in elderly age group and in females. A multimodality treatment approach is used to treat this entity and may improve survival compared with unimodality approach.

Obstacles of cancer survivorship: Sexuality issues - Need to break communication barriers

Many types of cancer and cancer therapies are frequently associated with sexual dysfunction. Previously sexual problems in cancer patients were mostly linked to breast or gynecologic cancer in females and prostate cancer in males. However, recent studies indicate that several other cancers are associated with sexual problems with estimates of sexual dysfunction ranging from 40% to 100% across various sites. Conversation related to sexual problems is not often the comfort zone for both patients and doctors. Numerous barriers contribute to this lack of communication, including lack of provider training, lack of time and lack of access to resources should the need arise to tackle the problem. Although clinicians often worry that patients will be offended or embarrassed if asked about sexual health, it is important to remember that multiple studies clearly indicate that patients want to talk about this topic with their doctors and that they desire more information about possible sexual side effects of treatment. It is essential that clinicians prepare patients for potential changes that may be encountered and let them know that discussion about sexual health concerns is welcome. Patients should be offered sexual counseling and informed about the availability of therapies for sexual dysfunctions. In this article, we aim to provide a concise review of the most common sexual problems experienced by survivors and discuss treatment options to overcome the problem.