Profile of 193 pediatric cancer patients managed with radiation therapy: Challenges and lessons learned.

Background: Series on radiotherapy (RT) practice in pediatric malignancies are limited in India as only a few centers practice pediatric RT, particularly under anesthesia. We aimed to study the clinical profile of pediatric cancer patients treated with RT and to analyze various challenges in pediatric RT under anesthesia. Materials and Methods: The data were prospectively maintained in Microsoft Excel spreadsheets. Pediatric cancer patients aged 0-14 years, registered in the RT department between February 1, 2019 and July 30, 2021were analyzed. Results: A total of 193 pediatric cancer patients (noncentral nervous system) received RT during the said period. Median age at presentation was 5.2 years (range: 9 months to 14 years) with a male-to-female ratio of 1.8:1. The majority of the patients were in the age group of 0-4 years (52.8%) followed by 5-9 years (29.5%) and ≥10 years (17.6%). Most common indications for RT included bone and soft-tissue tumors, retinoblastoma, Wilms tumor, neuroblastoma, and hematological malignancies. One hundred and seventy-nine (92.7%) patients received RT with curative intent, while 14 (7.3%) patients received palliative RT. Thirty (15.5%) patients needed anesthesia for RT. Ten (5.18%) patients required RT interruption due to toxicities with a median gap of 3 days. Conclusions: RT is challenging yet an important aspect of multidisciplinary care in paediatric cancers. Estimating the burden of pediatric patients in the RT department may help in assessing unmet needs, resource development, and prioritization, which may improve the cure rates.

Aliens in the thyroid gland: The secondary lesions.

BACKGROUND: A secondary lesion in the thyroid gland is a rare clinical scenario diagnosed preoperatively during the evaluation of a neck mass, postoperatively in a thyroidectomy specimen or in autopsy studies. Even though the thyroid gland is highly vascular, secondary malignant lesions are rare accounting for 0.2% of all thyroid malignancies. Thyroid gland secondary lesions are often metachronous in presentation as they are seldom evaluated in the initial diagnostic workup of the primary lesion. Fine-needle aspiration cytology (FNAC) is a useful modality for the diagnosis of secondary thyroid lesions. MATERIALS AND METHODS: A 6-year retrospective review (2016-2021) was carried out to assess the secondary lesions in the thyroid gland. Papanicolaou and field-stained FNAC smears of secondary thyroid lesions were reviewed. Ancillary techniques were performed on the cell block for differentiating from the primary thyroid gland lesions. RESULTS: There were 383 patients in our archives. There were only 18 cases (4.7%) that presented with secondary neoplastic lesions in the thyroid gland either by direct extension, metastases or as a hematolymphoid malignancy. There were 14 (77.7%) cases that presented with non-hematolymphoid secondary lesions while 4 (22.3%) cases presented with hematolymphoid malignancies. Thyroid secondaries were predominantly seen in female patients (female: male ratio of 1.5:1). Most of the cases presented with a synchronous secondary lesion (n = 14, 77.7%) and few metachronous secondary lesions were also noted (n = 4, 22.3%). CONCLUSION: Although exceedingly rare, the detection of secondary thyroid gland lesions is important for staging and planning treatment.

Mucoepidermoid carcinoma of the trachea in a 9-year-old male child: case report and review of literature.

Mucoepidermoid carcinoma (MEC) is most common malignancy of minor salivary glands in adults. Pulmonary MEC is extremely uncommon comprising of only 0.1%-0.2% of the primary lung malignancies and <1% of primary bronchial tumors. It is even rarer in children and literature limited to few case reports only. Here we present a case report of a 9-year-old boy diagnosed with primary MEC of trachea along with review of the literature. A 9-year-old male child presented with complaint of dry cough for two years which was later associated with shortness of breath after one year. Bronchoscopic examination revealed a growth arising from right lateral wall of carina occluding 50% of the lumen and detailed histopathological examination revealed it to be a MEC of the trachea. Patient underwent local excision of the tumor with primary anastomosis. In view of positive margins adjuvant radiotherapy of 60 Gy in 30 fractions were given to the tumor bed. Patient tolerated the treatment well and is disease free at 6 months follow-up. Experience with MEC of the trachea in children is limited and optimal treatment protocols have not been defined, with current treatment mainly extrapolated from MEC of the salivary glands.

Mismatch Repair Deficiency in Adult Granulosa Cell Tumors: an Immunohistochemistry-based Preliminary Study.

OBJECTIVE: Adult granulosa cell tumors (AGCTs) are rare ovarian malignant neoplasms; their etiopathogenetic mechanisms remain largely unelucidated. Lately, defects in mismatch repair (MMR) have been implicated in the pathogenesis of AGCTs. Demonstration of MMR deficiency in these tumors can help identify patients potentially eligible for immune checkpoint inhibition therapy. The present study was done to explore the role of MMR deficiency in the etiopathogenesis of AGCTs. METHODS: This was a retrospective study conducted on histopathologically confirmed AGCT cases. MMR protein expression was evaluated by immunohistochemistry (IHC) on tissue microarrays using an antibody panel of MSH2, MSH6, MLH1, and PMS2. RESULTS: Of a total of 40 ovarian AGCTs evaluated for MMR deficiency, none demonstrated loss of expression of any of the 4 MMR proteins. CONCLUSIONS: The results of our preliminary study show that there is no association between MMR deficiency with AGCT. Nevertheless, larger multicenter studies are needed to confirm or refute this observation.

What Did the Pandemic Teach Us About Palliative Radiation in Head and Neck Cancer?

Objective: To assess the feasibility and efficacy of palliative radiotherapy dose regimens for patients with locally advanced head and neck cancer. Methods: Fifty patients of previously untreated, inoperable, stage IVA and IVB squamous cell carcinoma of the head and neck, deemed unfit for radical treatment, were included in the study from May 2020 to June 2020. Two palliative radiotherapy regimens were used. First was a single fraction radiation with 8 Gy for patients with limited life expectancy and poor performance status, which was repeated after 4 weeks in case of good symptom relief. The second regimen was used for patients with good performance status and consisted of fractionated radiation with 30 Gy in 10 fractions over 2 weeks, which was followed by supplementary radiation with 25 Gy in 10 fractions over 2 weeks in patients with good symptomatic response at 2 weeks. Symptoms were assessed at baseline and at the end of 4 weeks after treatment completion using the numerical rating score. Patients were followed up for a median of 4.5 months and assessed for symptom control and overall survival. Results: Forty-eight patients completed treatment and were included for analysis. Of the 24 patients who received single fraction radiation, 13 (54.2%) were given the second dose. Improvement in pain and dysphagia were reported in 57.9% and 60% patients, respectively. A total of 55.5% noted decrease in size of the neck node. Twenty-four patients received fractionated radiation and 15 (62.5%) were given the second course after 2 weeks. Relief in pain and dysphagia was reported in 68.2% and 63.6% patients, respectively. There were no grade 3/4 toxicities. Symptom control lasted for at least 3 months in 30% of the patients who received single fraction radiation and 54.2% of the patients who received fractionated radiation. The estimated 6-month overall survival of the entire cohort was 51.4%. Conclusion: Judicious use of palliative radiation in advanced incurable head and neck cancers provides effective and durable symptom relief and should be used after careful consideration of patient prognosis, logistics of treatment, and goals of care.

Clinicopathological features of primary central nervous system diffuse large B cell lymphoma: Experience from a Tertiary Center in North India.

BACKGROUND: Primary central nervous system-diffuse large B-cell lymphoma (PCNS-DLBCL) is a rare extra-nodal Non-Hodgkin lymphoma. There is relative paucity of literature on PCNSL from Indian subcontinent. We aimed to analyze the clinicopathological features of PCNSL and categorize them into germinal center B cell (GCB) and non-GCB subtypes to assess their prognostic significance in Indian context. METHODS: All patients with histopathologically diagnosed PCNSLs at our center over a period of 6 years were recruited and classified into GCB and non-GCB using Han's algorithm (immunohistochemistry for CD10, BCL6 and MUM1). In situ hybridization (ISH) for Epstein-Barr virus (EBV)-encoded RNA was performed. RESULTS: Eighty-six cases of PCNS-DLBCL were included with median age of 55 years. Majority of them were supratentorial in location (n = 62). All patients were immunocompetent. On immunohistochemical assessment, 69 (80.2%) were of NGCB subtype, 10 (11.6%) were of GCB subtype, and 7 (8.1%) were unclassified. Overall, MUM1, BCL-6, and CD10 expressions were seen in 69 (80.2%), 28 (32.6%), and 2 cases (2.3%), respectively. Four cases (4.6%) showed C-MYC expression. The median overall survival (OS) was 675 days. None of the factors (age, sex, location, immunomarkers, and GCB vs. NGCB phenotype) showed correlation with OS; however, BCL6 positive cases showed slight better OS (P > 0.05). All cases were negative for EBV-LMP1 on ISH. CONCLUSION: The majority of the CNS DLBCL belongs to non-GCB phenotype and uniformly carry poor prognosis, irrespective of their phenotype. Individual markers, such as BCL-6, MUM1, or CD10, are unable to predict outcome in PCNS-DLBCL.

Prospective Study to Quantify Expansion Volumes Around the Involved Pelvic Lymph Nodes to Plan Simultaneous Integrated Boost in Patients With Cervical Cancer Undergoing Pelvic Intensity Modulated Radiation Therapy.

PURPOSE: This study aimed to calculate the expansion margins around enlarged pelvic lymph nodes to encompass internal motion and setup errors during intensity modulated radiation therapy with simultaneous integrated boost for cervical cancer. METHODS AND MATERIALS: Four-dimensional computed tomography scans were obtained for 19 patients with cervical cancer, and 32 fluorodeoxyglucose-avid pelvic lymph nodes were delineated in different respiratory phases to calculate respiratory displacement. Setup variations during daily treatments were estimated from on-board imaging. Descriptive statistics were used to quantify the expansion margins using Mc Kenzie and Van Herk formulas separately. An analysis of variance was used to analyze the volumetric impact of the margins. RESULTS: Based on the McKenzie formula, an internal margin (internal target volume) of 3 mm and setup margin (planning target volume) of 5 mm was required to adequately encompass respiratory and setup uncertainties, respectively. Although the use of the Van Herk formula necessitated a single 6 mm expansion margin for all uncertainties, which resulted in a significant reduction in boost volume, inherent limitations in our methodology might pose a higher risk of target miss with such smaller margins. CONCLUSIONS: An isotropic expansion margin of 3 mm for the internal target volume and 5 mm for the planning target volume is necessary for enlarged pelvic lymph nodes while planning intensity modulated radiation therapy with simultaneous integrated boost for cervical cancer.