Merkel cell carcinoma-A rare primary neuroendocrine skin tumor: case report and discussion.

Merkel cell carcinoma (MCC) is a rare but aggressive cutaneous primary small cell carcinoma with an unfavorable prognosis. It is a disease of the sun exposed skin of the elderly commonly involving the head, neck, and extremities. Though most cases present as localized disease, treatment should be definitive with wide excision of the primary lesion (2-3 cm margin) and prophylactic lymphadenectomy followed by irradiation to the primary site. Even when locoregional control is achieved, close surveillance is required due to high rates of local and systemic relapses. Chemotherapy is preserved for systemic disease, though the success of this treatment is limited and no chemotherapy protocol has been shown to improve survival.

Pericardial-peritoneal window: a novel palliative treatment for malignant and recurrent cardiac tamponade.

Transdiaphragmatic approach to the pericardium through a subxiphoid incision is a safe, rapid, and effective way to obtain drainage of the pericardium fluid in patient of disseminated malignancy with recurrent cardiac tamponade. No drainage tubes are needed; pericardial fluid is absorbed by the peritoneum; there is no need for double lumen tubes for single lung ventilation and the subxiphoid incisions are small and almost painless.

Osteosarcoma of breast: a rare case of extraskeletal osteosarcoma.

Primary osteogenic sarcomas of the breast are exceptionally uncommon. We describe such a case occurring in a 50 year-old woman who presented with a large painful mass in her left breast. Simple mastectomy of the left breast was performed. Microscopical and immunohistochemical findings established the diagnosis of primary osteogenic sarcoma. Similar to extremity osteosarcoma, adjuvant adriamycin and cisplatin based chemotherapy and external beam radiotherapy was given to the present case. She remained well 57 months later, without tumor recurrence. The current article made a literature search proving the rarity of this lesion type and discusses in detail the diagnostic implications and the treatment of this rare site tumor entity.

Aggressive multimodality management of locally advanced retromolar trigone tumors.

BACKGROUND: Retromolar trigone tumors are rare and aggressive malignancies. There is lack of quality evidence pertaining to their management due to the heterogeneity in treatment policies adopted. We retrospectively reviewed the patients of locally advanced retromolar trigone tumors treated with a standard and uniform multimodality management. METHODS: A retrospective review of patients with locally advanced retromolar trigone tumors was performed and an analysis of clinicopathologic profile, treatment details, and survival outcomes was carried out. RESULTS: Forty-two patients of locally advanced retromolar trigone tumors underwent the standard multimodality treatment. The majority of them presented with stage IVa disease. Margin negative resection could be achieved in 93% of patients. Histopathologically proven bone and node involvement was seen in 20 patients (47.6%) and 21 patients (50%), respectively. The 3-year disease-free and overall survival rates were 64% and 71%, respectively. CONCLUSIONS: Treatment of patients with locally advanced retromolar trigone tumors is challenging. However, good oncologic outcomes can be achieved by advocating an aggressive surgical approach with postoperative radiation therapy.

Locally advanced sebaceous cell carcinoma (T3) of eyelid: incidence and pattern of nodal metastases and combined modality management approach.

BACKGROUND: Sebaceous carcinoma (SbCC) is a rare malignancy that often mimics benign conditions. Lymphatic involvement, large T3 tumors herald a dismal survival for patients. We present our series of 13 cases of locally advanced SbCC of the eyelid treated at a surgical oncology unit and describe the clinical profile, patterns of nodal spread and recurrence pattern in this subset of SbCC. METHODS: A retrospective analysis of case records was carried out for patients presenting with orbital tumors between January 1997 and April 2010 in the department of Surgical Oncology, AIIMS, New Delhi, India. All patients underwent orbital exenteration and superficial parotidectomy with neck dissection was added to patients with clinically significant lymphadenopathy. All patients who underwent OE after 2002 were advised radiotherapy as adjuvant therapy. The end point was development of recurrence or end of two year follow up period which ever occurred earlier. RESULTS: Thirteen patients underwent orbital exenteration. Eleven patients had clinically palpable lymphadenopathy. Ten patients (76.9%) had pathologically confirmed metastatic nodes. Parotid lymph node involvement was present in all patients (100%); two of these ten patients also had level II b cervical lymph node involvement. Recurrence was observed in seven patients (53.8%). All recurrences were loco-regional only and no systemic metastases was seen. There were only two recurrences in the group that received PORT. CONCLUSIONS: Eyelid SbCC is a loco-regionally aggressive malignancy and adequate disease control can be achieved with combined modality approach of radical surgery followed by post operative radiotherapy.

Primary leiomyosarcoma of epididymis.

Primary leiomyosarcomas rarely arise from epididymis. But they are the most common histopathological types of sarcoma arising from the epididymis. Primary epididymal leiomyosarcoma occurs usually in older patients. We report a young patient of 35 years presenting with leiomyosarcoma of left epididymis. He did not have any metastasis and underwent left high inguinal orchiectomy. He is on regular follow-up and disease free for last two years.

Updates on the multimodality management of desmoplastic small round cell tumor.

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare and an aggressive malignancy with poor outcome. This tumor can co-express epithelial, neural, and mesenchymal markers. The molecular hallmark of DSRCT is the EWS-WT1 fusion protein. Despite the diversities in treatment modality, the best results have been seen with radical surgery and adjuvant or neoadjuvant chemotherapy.

Locally advanced salivary duct carcinoma of the parotid gland.

Due to the low incidence of salivary duct carcinoma (SDC), there is limited data in regard to the biologic behavior of this tumor, histopathological characteristics and its management. There is diversity in the management of parotid SDC. Various authors manage it with radical parotidectomy with or without neck dissection; others add adjuvant radiotherapy with radical surgery. The objective of the study is to see the biological behavior and management of the three patients with locally advanced SDC and review with the literature.

Basal cell adenocarcinoma of lacrimal gland.

Basal cell adenocarcinoma (BCAC) is a recently described rare salivary gland tumor. They are locally invasive and destructive tumors with rare incidence of metastasis. BCAC most commonly occur in the parotid gland followed by the submandibular and other minor salivary glands. The primary management of these tumors is surgery with or without adjuvant radiotherapy. Lacrimal gland is a very rare location of BCAC; only one case has been reported in English literature. We report a case of recurrent basal cell adenocarcinoma of lacrimal gland in a 75-year-old female. She had past history of local excision of a tumor in the lacrimal gland of same side 10 years back, details of which were not available with the patient. We discuss about the case and review the literature about treatment modality in basal cell adenocarcinoma.

A rare case of giant leiomyosarcoma in a filarial scrotum: a case report.

Giant leiomyosarcoma of scrotum is a rare tumour. A case of scrotum leiomyosarcoma is presented in a 67 year old patient with scrotal filariasis which was managed successfully with total scrotectomy with bilateral orchidectomy, degloved penis reconstructed with rotation advancement supra pubic fasciocutaneous flap. We made a literature search proving the rarity of this lesion type. Only 36 cases have been described and the first case in a filarial scrotum.

Ambulatory total extraperitoneal inguinal hernia repair: feasibility and impact on quality of life.

BACKGROUND AND OBJECTIVES: Feasibility of ambulatory laparoscopic inguinal hernia repair in developing countries is not known due to lack of dedicated outpatient centers. This study prospectively evaluated the feasibility of outpatient discharge after laparoscopic total extraperitoneal inguinal hernia repair done in combination with in-hospital services and its impact on quality of life. METHODS: Forty patients were studied who had uncomplicated inguinal hernias and fulfilled the selection criteria. Quality of life was evaluated by using the SF-12 questionnaire. RESULTS: Ninety percent of patients could be discharged as outpatients. Four patients required admission. No major complications or readmissions occurred. Physical components of quality of life deteriorated in the immediate postoperative period but improved to above preoperative levels within one month. A transient deterioration in subgroups of the mental health component was observed, which recovered to normal in less than a week. There was no significant alteration in the emotional component. There has been no recurrence at a median follow-up of 25 months. CONCLUSION: It was feasible to safely perform outpatient TEP in combination with routine in-hospital services without increasing complications or causing any adverse impact on quality of life. This was possible subject to adherence to proper selection and discharge criteria.