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Polymorphous low-grade adenocarcinoma (PLGA) is a rare neoplasm with an indolent course that occurs mainly in minor salivary glands and rarely in major salivary glands. It is characterised by morphological diversity hence the term polymorphous has been used. Diagnosis is often challenging due to variable microscopic growth patterns. The most common clinical presentation is asymptomatic painless mass which is slow-growing. The treatment of choice is wide surgical excision with negative margins. The role of radiotherapy is still not clear but considered in cases with positive margins and advanced stage. In this article, we review the clinical presentation, pathological features, treatment and prognosis of this rare entity. [ABSTRACT FROM AUTHOR]

Erectile dysfunction (ED) is one of the major but underreported concerns in cancer patients and survivors. It can lead to depression, lack of intimacy between the couple, and impaired quality of life. The causes of erectile dysfunction are psychological distress and endocrinal dysfunction caused by cancer itself or side effect of anticancer treatment like surgery, radiotherapy, chemotherapy and hormonal therapy. The degree of ED depends on age, pre-cancer or pre-treatment potency level, comorbidities, type of cancer and its treatment. Treatment options available for ED are various pharmacotherapies, mechanical devices, penile implants, or reconstructive surgeries. A complete evaluation of sexual functioning should be done before starting anticancer therapy. Management should be individualized and couple counseling should be an integral part of the anticancer treatment. [ABSTRACT FROM AUTHOR]

Introduction: Polymorphous low grade adenocarcinoma (PLGA) is a rare salivary gland neoplasm with an indolent course. It occurs primarily in the minor salivary glands but can rarely occur in the major salivary glands. It usually occurs in the fifth to seventh decades of life with female preponderance. Case Report: A 16-year-old male presented with recurrent painless swelling in the right preauricular region and with a history of surgical intervention at the same site in the past. His histopathology report was suggestive of pleomorphic adenoma. The swelling recurred after one year of excision and a superficial parotidectomy was performed. The detailed histopathological examination was suggestive of Polymorphous low grade adenocarcinoma. In view of close margins, the patient was given adjuvant radiotherapy. Thirty-three months post-surgery, he is alive and disease-free. We describe a rare case of PLGA of the parotid gland in a teenager with its clinical characteristics, histopathological features, and treatment. Conclusion: The occurrence of PLGA in the parotid gland is rare with only a few cases reported in literature. The diagnosis of PLGA is challenging due to morphological diversity. [ABSTRACT FROM AUTHOR]

Introduction: Urachal carcinoma is a rare malignancy of urogenital tract. The objective of this study was to assess the clinical presentation, histopathological findings, treatment and outcome of patients of urachal carcinoma at a tertiary care centre. Materials and Methods: A retrospective analysis of six cases of urachal carcinoma diagnosed over a period of 7 years from 2005 to 2011 was carried out. All pathologic specimens were reviewed by a single pathologist. Clinical and histological features along with treatment were reviewed and patient follow-up and survival outcome was obtained. Results: The mean age at diagnosis was 36 years. Of the six patients, five were male. The tumor was located in dome in five and dome and anterior wall in one patient. All patients underwent partial cystectomy with bilateral pelvic lymph node dissection. The Sheldon pathologic stage was stage II in 1, IIIA in 2, IVA in 3 cases. Five out of six patients received adjuvant radiotherapy. The mean follow-up period was 37 months. Three out of six were disease free at last follow-up. Conclusions: Urachal carcinomas are rare and usually locally advanced at presentation with a high risk of distant metastases. Surgery is the primary treatment of choice. Adjuvant therapy may decrease the chances of recurrence but it needs to be elucidated by prospective trials. [ABSTRACT FROM AUTHOR]

Primitive neuroectodermal tumors of the cervix are very rare. A 28-year-old pregnant woman presented with a cervical mass. The tumor was staged as IB2. The biopsy from tumor was suggestive of malignant small round cell tumor. She then underwent termination of pregnancy followed by radical hysterectomy. Based on morphologic and immunohistochemical profile, a diagnosis of peripheral primitive neuroectodermal tumor of the cervix was made. The patient received adjuvant chemotherapy and radiotherapy. The patient is alive and disease-free 33 months post-surgery. The present case highlights the importance of keeping primitive neuroectodermal tumors in the differential diagnosis of small cell neoplasms of the uterine cervix. Pregnancy should not be a barrier to early detection and treatment of this potentially aggressive tumor. The optimal treatment methods have not yet been established because of the rarity of the tumor. [ABSTRACT FROM AUTHOR]

Aim: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. There is limited published data on GIST from the Indian subcontinent. This 5-year retrospective analysis of 49 patients treated for GIST reports clinical and pathological features and survival outcome by risk stratification. Methods: We reviewed 49 cases of GIST from January 2004 to December 2008. Imatinib was administered after surgery in patients with either high-risk, residual or metastatic disease and at onset of recurrence or metastatic disease in patients with intermediate risk. Results: The mean age was 50 years (range, 17-80 years). Patients with localized tumor were classified as low ( n = 2), intermediate ( n = 4) and high risk ( n = 32), based on the primary tumor and mitotic index. At a median follow up of 21 months, 2-year and 3-year recurrence or progression-free survival rates were 61 and 39%, respectively, for all patients. The median recurrence-free survival rate in the intermediate-risk and high-risk groups were 7 and 49 months, respectively. The median progression-free survival in the residual ( n = 4) and metastatic disease group ( n = 7) was 10 and 29 months, respectively. Conclusion: This study demonstrates the role of imatinib in adjuvant and therapeutic settings. Responses have been durable and most patients tolerate the drug well at clinically effective doses. In view of high recurrence rates in the intermediate-risk group in our study, it would be better to keep these patients on strict follow up to detect recurrence at the earliest opportunity. [ABSTRACT FROM AUTHOR]

Primary malignant lesions of the vagina are uncommon, and vaginal sarcomas are even rarer. We describe a rare case of stage I, high-grade leiomyosarcoma of the vagina treated with combined modality treatment. A 39-year-old female presented with vaginal mass and underwent resection. Histopathological examination revealed atypical leiomyoma of the vagina with definite risk of recurrence. Eleven months later, she presented with a recurrent vaginal mass and underwent exploratory laparotomy with total abdominal hysterectomy and bilateral salpingo-oophorectomy plus resection of recurrent tumor and partial vaginectomy. The detailed histopathological examination was suggestive of leiomyosarcoma of the vagina. The patient received adjuvant radiotherapy and chemotherapy. The patient is alive and disease-free 29 months postsurgery. Experience with vaginal leiomyosarcomas is limited. The optimal treatment methods have not yet been established because of the rarity of the tumor. We add another case of leiomyosarcoma of the vagina to the limited existing literature. [ABSTRACT FROM AUTHOR]

Mesenchymal neoplasms of the larynx are rare and make up approximately 0.3% to 1.0% of all malignancies at this location. Low grade myofibroblastic sarcoma (LGMS) of larynx is a rare entity. We describe a rare case of LGMS of larynx who presented with complaint of hoarseness of voice. The patient was treated with total laryngectomy plus partial pharyngectomy followed by post-operative radiotherapy. Histopathologically, the lesion was composed of spindle cells that manifested variable cellular anaplasia and expressed smooth muscle actin (SMA) and focally S-100.One of the resection limits was involved so patient was given post-operative radiotherapy. The patient is alive and disease free 14 months after surgery. The characteristic clinical, histopathological features and treatment of this case are described with a literature review. [ABSTRACT FROM AUTHOR]

Alveolar soft part sarcoma (ASPS) is an enigma both for clinicians and pathologists. It is a rare soft tissue neoplasm affecting young adults. A 72-year-old female presented with a history of painful ulcer on the right side of the oral cavity associated with bleeding and difficulty in chewing. She underwent wide local excision of the lesion with segmental mandibulectomy and supraomohyoid neck dissection. Histopathological examination was suggestive of ASPS with involvement of resection margins. The patient developed rapid relapse after surgery and did not respond to chemotherapy and radiotherapy. She ultimately died of disease 10 months postsurgery. [ABSTRACT FROM AUTHOR]