Clinical outcome and morbidity in pediatric patients with nasopharyngeal cancer treated with chemoradiotherapy.

OBJECTIVES: The aim of the study was to evaluate the outcome and treatment-related morbidity in pediatric patients with nasopharyngeal carcinoma (NPC) treated with chemoradiotherapy. METHODS: We did a retrospective review of 41 pediatric patients diagnosed with NPC between 2000 and 2013. The majority of the patients were treated with neoadjuvant chemotherapy followed by concurrent chemoradiation with the dose of 70 Gy in 35 fractions. Eight patients were treated with intensity-modulated radiation therapy, while the remaining with three-dimensional conformal radiation therapy or two-dimensional simulation technique. RESULTS: The median age of the patients was 14 years (range 6-20 years). Most of the patients had locoregionally advanced disease (stage III/IVA/IVB). The histology of all the cases was undifferentiated carcinoma. Immunohistochemistry for the Epstein-Barr virus-Latent membrane protein 1 was positive in nine of the 13 tested cases. The median follow-up for all and the surviving patients was 26.6 months (range 2-140.8) and 51.2 months, respectively. The 3-year overall survival (OS) and event-free survival (EFS) rates were estimated at 83.7% (95% confidence interval [CI]: 64.8-93%) and 55.8% (95%CI: 38.7-69.8%), respectively. Distant metastases were the predominant pattern of failure. Treatment response showed an independent association with OS. T classification (T1/T2 vs. T3/T4) was significantly associated with EFS. Xerostomia, hypothyroidism, dental caries, neck fibrosis, trismus, and dysphagia were the common late effects in survivors. Radiation myelitis was observed in one patient. CONCLUSIONS: Treatment with neoadjuvant chemotherapy followed by concurrent chemoradiation provides good survival outcomes in pediatric NPC. The quality of life of the survivors is a pertinent area that necessitates consideration.

The role of brachytherapy in the treatment of squamous cell carcinoma of the head and neck.

Brachytherapy is a form of intensive local irradiation, allowing effective protection of surrounding structures with preservation of organ function and results in a favorable therapeutic ratio. It can be used alone, adjuvantly after surgery, and as a local boost in combination with external-beam radiation therapy. This paper is a literature review on the role of brachytherapy in the management of head and neck cancers with a special emphasis on papers published in the last 5 years. Technical details, effectiveness and potential toxicities of brachytherapy when used in different combinations with other therapeutic modalities and tumor sites are presented. Brachytherapy is an attractive treatment option in the management of primary malignancies and recurrent tumors in previously irradiated areas of the head and neck. It is effective and safe, and results in good functional and oncological outcomes.

Synchronous malignant mixed Müllerian tumor of the uterus with transitional cell carcinoma of the ovary.

A 55-year-old woman presented with a complaint of post-menopausal bleeding per vaginum. Local examination revealed a mass, protruding from the cervical os, which detached spontaneously. An adnexal mass was felt through the pouch of Douglas on per vaginum examination. Histopathological examination of the avulsed specimen revealed a diagnosis of malignant mixed Müllerian tumor. The patient underwent surgical staging with total abdominal hysterectomy, bilateral salpingo-oophorectomy, left pelvic lymphadenectomy, infracolic omentectomy, and peritoneal wash cytology. Pathological examination revealed a second primary tumor, that is, a transitional cell carcinoma of the ovary. Both the uterine malignant mixed Müllerian tumor and the ovarian transitional cell carcinoma were staged as IA. Subsequently, the patient was treated with adjuvant chemotherapy followed by radiotherapy. The patient is in complete remission at 1 year following the treatment. Synchronous genital tract neoplasms constitute a therapeutic challenge and necessitate an effective multimodality therapeutic approach based on meticulous pathological examination and tumor staging.

The Demographic and Clinicopathological Characteristics of Hepatopancreatobiliary Cancers From a Specialized Center in India.

Introduction Hepatopancreatobiliary (HPB) cancers are a distinct group requiring specialized multidisciplinary care. The present study was an analysis of HPB cancers. Methods The institutional data for two years from 2017 to 2018 was retrieved from the hospital-based cancer registry (HBCR) database in Excel format (Microsoft® Corp., Redmond, WA). The demographic details, method of diagnosis, tumor characteristics, and disease extent of the patients with HPB primary sites were retrospectively analyzed. Results Out of the 1417 patients with HPB malignancies, 1193 were analyzed. Most of the patients at our center hailed from North India. The distribution of HPB cancers was as follows: hepatocellular carcinoma (HCC) (n=717, 60.1%), gallbladder (GB) (n=230, 19.3%), periampullary carcinoma (n=76, 6.37%), head of the pancreas (HOP) (n=55, 4.61%), extrahepatic bile duct (EBD) (n=53, 4.44%), intrahepatic bile duct (IBD) (n=32, 2.68%), and body of the pancreas (BOP) (n=30, 2.52%). The most common disease presentation of HPB cancers was in the seventh and sixth decades of life. Male predominance was seen in all HPB cancers except in GB with a higher incidence in females. The predominant cause of chronic liver disease (CLD) with HCC was viral-related (53.7%) with hepatitis B virus (HBV) (34.3%) higher than hepatitis C virus (HCV) (19.2%) followed by non-alcoholic fatty liver disease (NAFLD)/non-alcoholic steatohepatitis (NASH) (24.8%) and then alcohol. Adenocarcinoma was the most common morphology in pancreatobiliary malignancies. The disease presentation was locoregional in 63.4% of HCC, 69.7% of periampullary carcinoma, and 50.9% of HOP cases. The patients with EBD and IBD cancers presented commonly with locoregional disease extent in 60.4% and 62.5%, respectively. Perihilar subsite was more commonly detected (71.7%) as compared to the distal one in the patients with EBD cancers. The patients with GB cancers (GBC) presented with distant metastases in 53.5% and locoregional disease in 33%. Distant metastases were present in 76.7% of the patients with BOP cancers. The liver was a common site of distant metastases in GB, periampullary, and pancreatic cancers. Conclusions The present study highlights the characteristics and the variations in disease presentation in different primary tumor sites of HPB cancers. In view of the common locoregionally advanced disease presentation of HCC, the patients with CLD need surveillance for the early detection of lesions. As the patients with HPB cancers show advanced disease presentation, effective locoregional and systemic therapies are needed.

Combined hepatocellular cholangiocarcinoma in hepatectomy specimens: A clinicopathologic analysis.

Background: Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is an uncommon form of primary liver carcinoma. It is heterogenous in terms of morphology, immunohistochemistry, radiology, and clinical features; making it a challenging entity for diagnosis. Aims: The purpose of the present study was to evaluate clinicopathological characteristics of patients with cHCC-CCA. Settings and Design: Retrospective observational study. Materials and Methods: The patients diagnosed with cHCC-CC were identified from hepatic surgical specimens and were evaluated. Statistical Analysis: Survival was estimated as per Kaplan-Meier method. Results: Out of six patients, five had undergone resection while one had liver transplant. Five were male and one was female and the mean age was 52 years. Tumor markers revealed raised serum alfa-fetoprotein and CA19.9 in four and three patients, respectively. Five of the liver specimens were cirrhotic. Diagnosis was predominantly based on tumor morphology. All cases were of Allen and Lisa type B and cHCC-CCA as per WHO (2019) classification. Stem cell features <5% were noted in two cases. Immunohistochemistry for programmed death 1/programmed death ligand 1 (PD1/PDL1) was negative in both the hepatocellular and cholangiocellular components in all six cases. Mismatch repair (MMR) protein expression was retained in two and deficient in four cases. The median follow-up after surgery was 21.3 months (range, 5-46.2 months). Five patients had intrahepatic and/or extrahepatic recurrence on follow-up after surgery. The median recurrence-free survival was estimated at 13.1 months (95% CI 5.67-20.6). Three patients had received salvage treatment. The median overall survival was estimated at 20 months (95% CI 0-45.3). Conclusions: The present study highlights the role of morphology in the diagnosis of cHCC-CCA. The choice of locoregional and/or systemic therapy after surgery may be individualized based on the clinicopathological characteristics.

External radiotherapy and brachytherapy in the management of extrahepatic and intrahepatic cholangiocarcinoma: available evidence.

This review aims to summarize the currently available evidence for the role of external radiotherapy and brachytherapy in the management of cholangiocarcinoma. High locoregional disease recurrence rates after surgical resection alone for both the extrahepatic cholangiocarcinoma (EHCC) and intrahepatic cholangiocarcinoma (IHCC) provide a rationale for using adjuvant radiotherapy with chemotherapy. We performed a literature search related to radiotherapy in cholangiocarcinoma published between 2000 and 2016. The role of radiation is discussed in the adjuvant, neoadjuvant, definitive and the palliative setting. Evidence from Phase II trials have demonstrated efficacy of adjuvant chemoradiation in combination with chemotherapy in EHCC. Locally advanced cholangiocarcinoma may be treated with neoadjuvant chemoradiotherapy. In the case of downsizing, assessment for resection may be considered. Brachytherapy offers dose escalation after external radiotherapy. Selected unresectable cases of cholangiocarcinoma may be considered for stereotactic body radiation therapy with neoadjuvant and/or concurrent chemotherapy. Liver transplantation is a treatment option in selected patients with EHCC and IHCC after neoadjuvant chemoradiation. Stenting in combination with palliative external radiotherapy and/or brachytherapy provides improved stent patency and survival. Newer advanced radiation techniques provide a scope for achieving better disease control with reduced morbidity. Effective multimodality treatment incorporating radiotherapy is the way forward for improving survival in patients with cholangiocarcinoma.

Recurrent sebaceous carcinoma of the eyelid: Outcome after postoperative reirradiation.

BACKGROUND: The purpose of this study was for us to describe a case of recurrent sebaceous carcinoma treated with postoperative reirradiation. METHODS: A 38-year-old man was diagnosed with sebaceous carcinoma of the right lower eyelid. The patient developed local recurrence 4 times, with the first one at 30 months after the excision. The first local recurrence was treated with excision and postoperative radiotherapy with 60 Gy/30 fractions/6 weeks. He manifested preauricular nodal metastasis with the third local recurrence, which was confirmed with (18)F-fluorodeoxyglucose positron emission tomography-CT ((18)F-FDG PET-CT). He received 2 courses of adjuvant reirradiation to the right orbit with 45 Gy/25 fractions/5 weeks and 30 Gy/15 fractions/3 weeks, respectively. RESULTS: The patient was successfully treated with no evidence of locoregional recurrence at 2 years after the cancer-directed therapy. The patient's follow-up from the date of diagnosis has been 9 years. CONCLUSION: Adjuvant reirradiation with modest doses may be considered with a view to provide disease control and long-term survival.

Solitary fibrous tumor of the sellar region treated with adjuvant radiation therapy.

The solitary fibrous tumor of central nervous system is rare. Herein, a case of solitary fibrous tumor arising from sellar region is described. A 60-year-old man underwent subtotal excision of the tumor because of extensive infiltration of optical and vascular structures. In view of the presence of residual tumor, he was treated with adjuvant radiation therapy. After a follow-up period of 1 year, there was no progression of the lesion evident on magnetic resonance imaging of the brain. Solitary fibrous tumor should be considered as one of the differential diagnosis of a mass lesion arising in sellar region. Immunohistochemistry with CD34 is valuable for discerning the diagnosis. Complete surgery should be the goal of treatment and adjuvant radiation therapy may be considered for residual or recurrent disease.

Extrapulmonary Small Cell Carcinoma - a Case Series of Oropharyngeal and Esophageal Primary Sites Treated with Chemo-Radiotherapy.

BACKGROUND: The optimal sequence and extent of multimodality therapy remains to be defined for extrapulmonary small cell carcinoma because of its rarity. The purpose of our study was to assess the response to neoadjuvant chemotherapy followed by chemoradiation/radiation in patients with extrapulmonary small cell carcinoma. MATERIALS AND METHODS: Four consecutively diagnosed patients were included in this study. The primary tumor site was oropharynx in three patients and esophagus in one. The patients with the limited disease were treated with chemotherapy followed by concurrent chemoradiation (n=2) or radiotherapy (n=1). The patient with the extensive disease with the primary site in vallecula was treated with chemotherapy and palliative radiotherapy to the metastatic site. RESULTS: The median follow-up was 22.5 months (range, 8-24 months). Three patients with the limited disease (base of tongue, n=2; esophagus, n=1) were in complete remission. The patient with the extensive disease died of loco-regional tumor progression at 8 months from the time of diagnosis. CONCLUSIONS: The combination of chemotherapy and radiotherapy is the preferred therapeutic approach for patients with extrapulmonary small cell carcinoma. Induction chemotherapy followed by concurrent chemoradiation or radiation provides a good loco-regional control in patients with limited disease.

Malignant peripheral nerve sheath tumour of the maxilla.

A 38-year-old man was diagnosed with malignant peripheral nerve sheath tumour of the maxilla. He was treated with total maxillectomy. Histopathological examination of the resected specimen revealed a close resection margin. The tumour was of high grade with an MIB-1 labelling index of almost 60%. At six weeks following the surgery, he developed local tumour relapse. The patient succumbed to the disease at five months from the time of diagnosis. The present report underlines the locally aggressive nature of malignant peripheral nerve sheath tumour of the maxilla which necessitates an early therapeutic intervention. A complete resection with clear margins is the most important prognostic factor for malignant peripheral nerve sheath tumour in the head and neck region. Adjuvant radiotherapy may be considered to improve the local control. Future research may demarcate the role of targeted therapy for patients with malignant peripheral nerve sheath tumour.

Institutional experience of interstitial brachytherapy for head and neck cancer with a comparison of high- and low dose rate practice.

AIMS: To describe our institutional experience with high dose rate (HDR) interstitial brachytherapy (IBT) compared with previously reported results on the low dose rate (LDR) practice for head and neck cancer. MATERIALS AND METHODS: Eighty-four patients with oral cavity (n=70) or oropharyngeal cancer (n=14) were treated with 192Ir HDR-IBT. Seventy-eight patients had stage I or II tumour. The patients treated with IBT alone (n=42) received 39-42 Gy/10-14 fractions (median=40 Gy/10 fractions). With respect to the combination therapy group (n=42), prescription dose comprised of 12-18 Gy/3-6 fractions (median=15 Gy/5 fractions) for IBT and 40-50 Gy/20-25 fractions (median=50 Gy/25 fractions) for external radiotherapy. Brachytherapy was given as 2 fractions per day 6 hours apart with 4 Gy per fraction for monotherapy and 3 Gy per fraction for combination therapy. RESULTS: Four patients were not evaluable in the analysis of outcome. The primary site relapse rates were 23.8% (10/42) and 68.4% (26/38) in patients treated with IBT alone and combination therapy, respectively (p<0.001). Salvage surgery was performed in 19 patients. The 5-year local control rate was estimated at 62% and the disease-free survival (DFS) rate at 52% for all patients. Local control with respect to T1 and T2 tumours was 84% and 42%, respectively. CONCLUSIONS: Our present series on HDR-IBT and the previous report on LDR-IBT for head and neck cancer demonstrated similar DFS rates at 5 years (52%). The rate of regional failure in node-negative patients was <20% in both of our series. HDR-IBT offers similar results to LDR-IBT for head and neck cancer.

Recurrent sebaceous carcinoma of the scalp in a young male treated with adjuvant radiotherapy.

Sebaceous carcinoma is a rare malignancy of the skin appendages. It tends to occur in elderly patients. Orbital region is the most commonly involved site seen in 75% of cases. The involvement of extra-orbital sites is infrequent. Herein, a case of extra-ocular sebaceous carcinoma arising in the scalp of a 20-year-old man is described.The patient developed tumor relapse after excision biopsy. He was treated with wide local excision of the tumor. However, the patient developed local recurrence after an interval of four months for which he again underwent wide local excision. He did not manifest any regional or distant metastases. In view of the locally aggressive tumor, he received adjuvant radiation therapy. The patient was successfully treated with no evidence of any local recurrence seen after a follow-up period of one year. The timely recognition of sebaceous carcinoma is imperative so as to execute the primary treatment i.e., wide local excision. Adjuvant radiotherapy may be considered to improve the clinical outcome for recurrent tumors.

Treatment outcome of patients with carcinoma of vulva: experience from a tertiary cancer center of India.

PURPOSE: The aim of our retrospective study was to analyze and report the clinical outcome of patients with vulvar carcinoma (VC) treated at our center. MATERIALS AND METHODS: We retrieved the information regarding patients' clinical details, treatment given, survival and complications from the case records of all VC patients who were treated at our center during the year 1998-2005. Overall survival (OS) was determined with respect to age, histopathological grade, stage of disease, treatment group, pathological lymph node status, etc. RESULTS: A total of 60 case records were retrieved for this retrospective analysis. Age ranged from 24 to 92 years (median 63 years). International Federation of Gynecology and Obstetrics (FIGO) stage distribution was as follows: stage I: 2 patients; stage II: 17 patients; stage III: 31 patients; stage IV: 9 patients; and unknown stage: 1 patient. Thirty-three patients underwent surgery (wide local excision 3, radical vulvectomy 30). Eleven patients received postoperative radiation therapy (PORT), 12 received palliative radiation therapy (RT) and 15 underwent definitive RT (5 of them received concurrent chemotherapy). Median follow-up period was 23 months (range 2-144 months). The 5-year OS for all stages was 41%. FIGO stage and pathological node positivity were found to be statistically significant prognostic factors for survival. CONCLUSION: Despite the majority of patients presenting in advanced stage, the 5-year OS of 41% in our series reflects a decent therapeutic outcome. The results have shown FIGO stage and pathological node positivity to be significant prognostic factors for survival. The use of preoperative chemotherapy/RT needs to be studied in our setup.