Epithelial myoepithelial carcinoma: A case report.

ABSTRACT: Epithelial myoepithelial carcinoma is a rare tumor with the parotid gland being the most common primary site of origin. The tumor may also very rarely originate in minor salivary glands of the soft palate. Due to the rarity of its occurrence and histogenesis, clear-cut therapeutic guidelines are not defined. The present report describes the case of a 56-year-old female patient who was diagnosed to have epithelial myoepithelial carcinoma (EMC) of the minor salivary gland of the soft palate, Stage T2 N0 M0 (Stage group II). The patient was treated with radical radiotherapy followed by adjuvant chemotherapy and is alive with no evidence of disease after 12 months following the end of treatment.

Cutaneous metastasis in bladder cancer.

Cutaneous manifestation of cancers is a rare occurrence, even more so for bladder cancer, with a limited number of published reports. Iatrogenic implantation has been a major cause for it. With no clear distinction pattern from other common dermatological lesions, scattered occurrence, and poor survival; no definitive strategies are present for the management of these dermal manifestations. The present article describes a case of scalp lesion consistent with metastatic urothelial carcinoma along with a review of the literature.

Carcinosarcoma of buccal mucosa: A case report.

Carcinosarcoma is an extremely rare variant of squamous cell carcinoma characterized by biphasic histology defined by epithelial and mesenchymal components. Because of the aggressive nature, early risk of metastasis, and high mortality, this tumor is associated with poor prognosis. Surgery is considered as the main modality of treatment, although radiotherapy can be discussed in inoperable cases. The present paper describes a rare case of carcinosarcoma of buccal mucosa.

Extracorporeal irradiation in malignant bone tumors: Single institution experience and review of literature.

In the last couple of decades, the management of malignant bone tumor (MBT) has seen a sea change. With the advent in surgical technics, radiation therapy, and chemotherapy, it has moved from disabling amputation to limb salvage surgery. Extracorporeal irradiation (ECI) and re-implantation of resected bone is a useful method of limb salvage of MBTs. In our study, we analyzed and presented the results of 8 cases of MBTs treated with this modality. Between 2014 and 2017, 8 patients with primary MBT were enrolled for ECI technique who are meeting the eligibility criteria. Before taking the patient for ECI treatment, a multispecialty tumor board discussion was done for each patient. All of them received neo-adjuvant and adjuvant chemotherapy except the patients with histology of giant cell tumor. Following neoadjuvant chemotherapy bone excision surgery was performed, and the resected bone was taken for ECI with the dose of 50 Gray in a single fraction. After ECI, bone segment was re-implanted at osteotomy site in the same setting. After completion of adjuvant chemotherapy, the patients were then followed up for any sequelae, local and systemic control, ambulation, and functional outcome. Out of 8 patients, there were 5 males and 3 females with mean age of 22 (range 13-36). The involved bone was the tibia in 6 patients, ischium in 1 patient, and femur in 1 patient. Histopathologically, the malignancies included 3 osteosarcoma, 3 Giant cell tumor, 1 Ewing's sarcoma and 1 chondrosarcoma. At median follow-up of 12 months (range 6-26 months), local control rate was 87.5% and systemic control rate was 75%. Perioperative ECI and re-implantation is a useful, convenient, and inexpensive technique. The overall treatment time is reduced. The patient's own bone fits perfectly to the resection site with reduced risk of graft site infection. The risk of local recurrence due to tumor re-implantation is negligible with tumoricidal radiation doses of ECI, and it is usually associated with manageable sequelae. Recurrence rates are acceptable and salvageable with surgery.

Epidemiological characteristics of endometrial cancer patients treated at a tertiary health center in National Capital Territory of India.

Settings and Design: A retrospective study which analyzed the data of female patients attending a tertiary care center in National Capital Territory for the treatment of endometrial cancer. Materials and Methods: Eighty-six histopathologically confirmed cases of carcinoma endometrium were taken from January 2016 to December 2019. Detailed information was collected regarding patient's case history, sociodemographic data (age of presentation, occupation, religion, residence, and substance addiction), clinical presentation, diagnostic and therapeutic procedures, and known risk factors (age at menarche and menopause, parity, obesity, use of oral contraceptive pills, hormone replacement therapy, and comorbidities such as hypertension and diabetes). Statistical Analysis Used: After analysis, results were presented as mean ± standard deviation and frequency. Results: Eighty-six percent of the patients (n = 73) were in the age group of 40-70 years; the mean age of the patients at diagnosis of endometrial cancer was 54 years. Eighty-one percent (n = 70) of the patients were from urban areas. Sixty-seven percent of the females (n = 54) were Hindu. All the patients were housewives with nonsedentary lifestyles. Most patients (88%; n = 76) presented with bleeding per vaginum. Fifty-nine percent (n = 51) had stage I disease, followed by 15% (n = 13) with stage II, 14% (n = 12) with stage III, and 12% (n = 10) with stage IV disease. Eighty-two percent (n = 72) of the patients had endometrioid carcinoma. Other less common variants were mixed Mullerian malignant tumor, squamous, adenosquamous, serous, and endometrioid stromal. Forty-four percent (n = 38), 39% (n = 34), and 16% (n = 14) of the patients had grade I, grade II, and grade III tumor, respectively. 53.5% of the cases (n = 46) had >50% myometrial invasion at the time of presentation. Eighty-two percent (n = 71) of the patients were postmenopausal. The mean age at menarche and menopause was 13 years and 47 years, respectively. Fifteen percent (n = 13) of the females were nulliparous. Forty-six percent (n = 40) of the patients were overweight. Most patients (82%) had no history of addiction. Twenty-five percent (n = 22) of the patients had hypertension, and 27% (n = 23) has diabetes as comorbidity. Conclusions: The incidence of endometrium cancer is showing a steady rise in the recent past. Early age of menarche, late age of menopause, nulliparity, obesity, and diabetes mellitus are well-documented risk factors for uterine cancer. Better outcome and control of disease is possible by understanding of endometrial cancer etiology, risk factors, and its preventive measures. Thus, a robust screening program is warranted to detect the disease in early stage and for increased survival.

Systematic Review and Individual Patient Data Analysis of Uncommon Variants of Glioblastoma: An Analysis of 196 Cases.

Objectives: Different variant of GBM has been reported viz. Epithelioid Glioblastoma (GBM-E), Rhabdoid GBM (GBM-R), Small cell GBM (GBM-SC), Giant cell GBM (GBM-GC), GBM with neuro ectodermal differentiation (GBM-PNET) with unknown behavior. Materials: We conducted a systematic review and individual patient data analysis of these rare GBM variants. We searched PubMed, google search, and Cochrane library for eligible studies till July 1st 2016 published in English language and collected data regarding age, sex, subtype and treatment received, Progression Free Survival (PFS), Overall Survival (OS). Statistical Package for social sciences (SPSS) v16 software was used for all statistical analysis. Results: We retrieved data of 196 patients with rare GBM subtypes. Among these GBM-GC is commonest (51%), followed by GBM-R (19%), GBM-PNET (13%), GBM-SC (9%) and GBM-E (8%). Median age at diagnosis was 38, 40, 43.5, 69.5 and 18 years, respectively. Male: female ratio was 2:1 for GBM-E, and 1:3 for GBM-SC. Maximal safe resection followed by adjuvant local radiation was used for most of the patients. However, 6 patients with GBM-PNET, 3 each of GBM-E, GBM-SC received adjuvant craniospinal radiation. Out of 88 patients who received chemotherapy, 64 received Temozolomide alone or combination chemotherapy containing Temozolomide. Median PFS and OS for the entire cohort were 9 and 16 months. In univariate analysis, patient with a Gross Total Resection had significantly better PFS and OS compared to those with a Sub Total Resection [23 vs. 13 months (p-0.01)]. Median OS for GBM PNET, GBM-GC, GBM-SC, GBM-R and GBM-E were 32, 18.3, 11, 12 and 7.7 months, respectively (P = 0.001). Interestingly, 31.3%, 37.8% of patients with GBM-E, GBM-R had CSF dissemination. Conclusion: Overall cohort of rarer GBM variant has equivalent survival compared to GBM not otherwise specified. However, epithelioid and Rhabdoid GBM has worst survival and one third shows CSF dissemination.

Demography, Pattern of Care, and Survival in Patients with Xanthoastrocytoma: A Systematic Review and Individual Patient Data Analysis of 325 Cases.

Objectives Xanthoastrocytoma (XA) is a low-grade glial tumor seen in young adults and there is lack of robust data on treatment of this rare tumor. In this systematic review and individual patient's data analysis, we aimed to look into the demography, pattern of care, survival outcomes, and prognostic factors in patients with both Grade II and III XA. Methods A comprehensive search was conducted with the Medical Subject Heading terms: "Xanthoastrocytoma; Pleomorphic Xanthoastrocytoma; Anaplastic Xanthoastrocytoma; Xanthoastrocytoma AND treatment; and Anaplastic Xanthoastrocytoma AND survival" to find all possible publications. Results A total of 325 individual patients from a total of 138 publications pertaining to XA were retrieved. Median age of the entire cohort was 19 years. About 56.1% of the patients underwent a gross total resection (GTR) and 31.4% underwent a subtotal resection. Nearly, 76.6% of the patients had a Grade II tumor and adjuvant radiation was delivered in 27.4% of the patients. Estimated 2- and 5-year progression-free survival (PFS) were 68.5 and 51.2%, respectively. Age, grade, and extent of surgery were significant factors affecting PFS. Estimated 2- and 5-year overall survival (OS) was 88.8 and 78%, respectively. The median OS for Grade II and Grade III tumors were 209 and 49 months, respectively. Age and extent of surgery were significant factors affecting OS. Conclusion XA is a disease of young adults with favorable prognosis. Younger patients (<20 years), patients who undergo a GTR, and patients with a lower grade tumor have a better treatment outcome.

Grade II Pleomorphic Xanthoastrocytoma; a meta-analysis of data from previously reported 167 cases.

Pleomorphic Xanthoastrocytoma [PXA] is a rare low grade glial tumor commonly affecting young adults. We did this systematic review and meta-analysis to identify prognostic factors and optimal treatment in these patients. A thorough search of the PubMed, Google scholar was made to find all possible publications related to grade II PXA. A total of 167 patients from 89 articles were included in the analysis. Median age of the entire cohort was 20 years. Headache was the most common presentation in 49.1% of the patients followed by seizure in 27.9%. Temporal lobe was the most common location of the tumor. 63% patents underwent a gross total resection [GTR] and 26.7% underwent a sub total excision [STR]. Adjuvant radiation was given to 17.6% of patients. Median follow-up for the entire cohort was 33 months. Estimated median overall survival [OS] for the entire cohort was 209.0 months [96% CI: 149.7-268.3]. Estimated median progression free survival [PFS] was 48 months [95% CI: 31.9-64.0]. In univariate and multivariate analysis younger patients and patients who underwent a GTR had a significantly better survival outcome. Use of adjuvant therapy was not found to be a significant factor affecting PFS or OS. Radiotherapy was used in salvage treatment in 76.1% of the patients. Younger patients and patients who undergo a GTR, have better survival outcomes. There is inadequate evidence to recommend routine adjuvant radiation or chemotherapy in all patients with grade II PXA.

Impact of surgery, adjuvant treatment, and other prognostic factors in the management of anaplastic ganglioglioma.

BACKGROUND/PURPOSE: Anaplastic ganglioglioma (AGG) is a rare tumor with both glial and neuronal component accounting for less than 1% of all CNS tumors with limited information about the optimum treatment and outcome of these tumors. METHOD AND MATERIALS: We did a thorough search of the PubMed with the following MesH terms: "Ganglioglioma; Anaplastic ganglioglioma; Ganglioglioma AND treatment; and Anaplastic ganglioglioma AND survival" to find all possible publications related to AGG to perform an individual patient data analysis and derive the survival outcome and optimum treatment of these tumors. RESULTS: A total of 56 articles were retrieved pertaining to AGG with 88 patients. However, a total of 40 publications found eligible with 69 patients for individual patient data analysis. Median age for the entire cohort was 16 years (range 0.2-77 years). Surgical details were available for 64 patients. A gross total or near total resection was reported in 21 cases (32.8%), subtotal resection or debulking was reported in 25 cases (39.1%). Surgical details were available for 64 patients. A gross total or near total resection was reported in 21 cases (32.8%), and subtotal resection or debulking was reported in 25 cases (39.1%). Median overall survival (OS) was 29 months [95% CI 15.8-42.2 months] with 2- and 5-year OS 61 and 39.4% respectively. CONCLUSION: AGG is associated with a dismal. Pediatric age and a gross total resection of tumor confer a better progression-free survival and OS. Hence, surgery should remain the cornerstone of therapy. However, because of modest survival, there is enough opportunity to improve survival with addition of adjuvant radiation and chemotherapy. A whole genome sequencing and molecular characterization would help to derive the best treatment option.

Effect of Surgery, Adjuvant Therapy, and Other Prognostic Factors on Choroid Plexus Carcinoma: A Systematic Review and Individual Patient Data Analysis.

PURPOSE: The optimal treatment of patients with choroid plexus carcinoma (CPC) is unclear. We conducted a systematic review and meta-analysis of individual patient information to determine the effect of surgery, adjuvant therapy, and other prognostic factors for CPC. METHODS AND MATERIALS: A comprehensive search of the PubMed and Google Scholar databases was performed using the following MeSH terms to find all possible reports on CPC: choroid plexus tumor; choroid plexus carcinoma; choroid plexus carcinoma AND treatment; and choroid plexus carcinoma AND survival. We performed an individual patient data analysis to assess the strength of the potential associations between different variables and the outcomes for patients with CPC. RESULTS: Data from 284 patients were retrieved from 89 studies. The median patient age was 2 years, with 26% patients diagnosed in the first year of their life. Of these 284 patients, 52.8% had undergone gross total resection (GTR) or near total resection. The median follow-up period for the entire cohort was 10.8 months. The median progression-free survival (PFS) was 13 months (95% confidence interval 8.14-17.8). PFS was better for patients >aged 5 years and those who had undergone GTR. The median overall survival (OS) was 29 months (95% confidence interval 16.3-41.7). OS was better for patients aged >5 years, those who had undergone GTR, those who had received adjuvant treatment, and those with a parenchymal tumor site. CONCLUSIONS: CPC is an aggressive tumor, with a median PFS of 13 months and median OS of 29 months. All patients should undergo maximal safe resection, because GTR is associated with improved survival. The use of adjuvant radiation and chemotherapy were also associated with improved outcomes.

Patterns of care and survival outcomes in patients with astroblastoma: an individual patient data analysis of 152 cases.

BACKGROUND: Astroblastoma (AB) is a rare tumor with significant dilemma regarding diagnostic criteria, behavior, and optimum treatment. MATERIALS AND METHODS: We searched PubMed, Google Search, and Cochrane Library for eligible studies with the following search words: astroblastoma, high-grade astroblastoma, and anaplastic astroblastoma till July 1, 2016, published in English language and collected data regarding age, sex, site of disease, pathological grade, treatment received, and survival. RESULTS: Data of 152 patients were retrieved from 63 publications. Median age was 16 years (range 0-71). Females were affected twice more frequently than male (70.3 vs. 29.7%). Tumors were most commonly located in the frontal (39%) followed by parietal lobe (26.7%). Fifty-two and 25% of the patients had headache and seizure at presentation, 76.3% of the patients underwent a gross total resection, 41 out of 89 had a high-grade tumor, and 56 patients received adjuvant radiation with a median dose of 54 Gy (range 20-72). Adjuvant chemotherapy was used in 23 patients. Temozolomide was the most common drug used in 30% of the patients. A combination of cisplatin, etoposide with vincristine, or ifosfamide was used in 17%. Median follow-up duration was 37 months (range 1-238). Median progression-free survival and OS were 36 and 184 months, respectively. Patients with a higher-grade tumor had significantly worse OS with HR 5.260 and p = 0.001. Forty patients experienced local progression. Sixty-five percent patients underwent surgery while 50% underwent radiation as salvage. CONCLUSION: AB has two distinct grades with higher-grade tumors having significantly poor survival. Maximal safe surgery followed by adjuvant radiation and temozolomide should be advocated for these tumors.

Porocarcinoma scalp with high risk features treated with surgery and adjuvant radiotherapy: A case report and review of literature.

Eccrine porocarcinoma is a rare malignant sweat gland tumor arising from the intra dermal part of the gland and accounts for only 0.005% of all epithelial cutaneous tumors. Commonly involved site includes extremities and face. Scalp is a rare site for porocarcinoma with less than 20 reported cases so far. Wide local excision with clear margins remains the treatment of choice. Review of literature revealed a local recurrence rate of 37.5% and a nodal involvement risk of 20%. Porocarcinoma of the scalp is peculiar in that the primary tumor may be large at presentation, making surgery with adequate margins difficult. Adjuvant radiotherapy must be considered in a case to case basis due to the high local recurrence rates compared to other sites of porocarcinoma and should be given to all patients with close margins and extra capsular extension.