Primary malignant giant cell tumor of the sternum

Primary malignant giant cell tumor (PMGCT) is a diagnosis based on the presence of a high-grade sarcomatous component along with a typical benign giant cell tumor (GCT). We report the first case of PMGCT of the sternum in a 28-year-old male with painless swelling over the manubrium sterni. The differential diagnoses of PMGCT and giant cell-rich osteosarcoma were considered. Surgical resection was performed, and the reconstruction was done with a neosternum using polymethyl methacrylate and prolene mesh. At 30 months follow-up, the patient is disease-free.

Warthin-like mucoepidermoid carcinoma of the parotid gland: a diagnostic and therapeutic dilemma

Mucoepidermoid carcinoma (MEC) is the most common malignant salivary gland tumor. Although the parotid gland is the most common site of involvement, other major salivary glands and the minor salivary glands­most commonly of the palate­also can be involved. The management of mucoepidermoid carcinoma depends on the grade of the tumor and the adequacy of resection. We present the case of a 56-year-old female presenting a painless progressive cheek mass over 2 months. Imaging and fine-needle aspiration cytology provided the diagnosis of Mucoepidermoid carcinoma. A superficial parotidectomy was done, and the histopathology revealed a predominantly cystic tumor with a bilayered epithelium of oncocytic and basal cells. Moderate nuclear pleomorphism with infiltration of atypical squamous cells in few glandular cysts was seen. Special staining revealed the presence of intracellular mucin. A diagnosis of Warthin-like variant of MEC was made, based on these findings. After the surgical procedures, the patient is disease-free at 8 months of follow-up. The Warthin-like variant is a rare variant of MEC with fewer than 10 cases described in the English literature. Various differential diagnoses include the malignant transformation of Wartin tumor (WT), squamous metaplasia of WT, and metastasis from a distant primary. We emphasize the role of routine microscopy in identifying rare variants of common malignancies. Even though translocation studies are helpful in diagnosis, the typical histopathological findings should confirm it.

Pleomorphic hyalinizing angiectatic tumor of the mesorectum: a rare case and review of literature

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of uncertain tissue origin. Although it has been classified as a benign tumor under the WHO classification, it is locally aggressive, and multiple recurrences have been reported. PHAT commonly involves the lower extremities; however, various unusual sites of origin have been reported. We present the case of a 30-year-old female with dysmenorrhea, who presented a presacral mass on imaging. The core biopsy confirmed the diagnosis of PHAT. She underwent laparotomy and excision. Histopathology confirmed the presence of a tumor comprised of aggregates of ectatic vessels with perivascular hyalinization. An immunohistochemical study showed diffuse CD34 positivity, but S100, MDM2, and smooth muscle actin negativity. After surgical procedures, the patient is disease free as at the 12-month follow-up. Only 120 cases have been published in the English literature to date. Our study is only the third case of PHAT arising from the pelvis to be reported. Though considered to be a rare condition, the diagnosis of PHAT should always be considered in the differential diagnosis of well-defined hypervascular soft tissue mass in the pelvis. The typical histopathological findings along with immunohistochemistry should clinch the diagnosis.

Primary colonic liposarcomatosis: report of a case with review of literature

The colon is a rare site of occurrence of liposarcoma, as either the primary site or by secondary involvement from a retroperitoneal liposarcoma. Liposarcomatosis denotes simultaneous occurrence of multiple liposarcomas. There are only 17 cases of primary colonic liposarcoma reported in the English literature­one of which was primary colonic liposarcomatosis. We depict the second case of primary colonic liposarcomatosis in a 57-year-old female who presented with abdominal swelling and pain. On exploratory laparotomy, two large masses were seen arising from the wall of the right colon along with multiple smaller masses attached to the colon. Right hemicolectomy with en bloc excision of the masses was performed along with hysterectomy and pelvic floor repair. Macroscopically, multiple exophytic masses and one endophytic mass were identified. The exophytic masses were of variable size and were found to hang from the colon by a thin pedicle simulating variable-sized appendices epiploicae. Histopathologically, the lesions showed the morphology of well-differentiated liposarcoma. This appears to be a case of primary colonic liposarcomatosis. There is only one other similar case reported in the English literature, to the best of our knowledge.

Organ-preserving multimodality management of squamous cell carcinoma of anal canal.

AIM: To study the efficacy of an organ-preserving, sequential chemoradiation therapy for squamous cell carcinoma of the anal canal, and of salvage surgery in those in whom this treatment fails. METHODS: Forty biopsy-proven untreated patients (28 men) with squamous cell carcinoma of the anal canal received two cycles of chemotherapy using cisplatin and methotrexate, followed by 45 to 60 (median 50) Gy external beam radiotherapy. Salvage surgery was offered to those in whom this treatment failed. Overall survival, disease-free survival and colostomy-free survival were analyzed. RESULTS: Most patients (n=35; 87%) had T3 or T4 lesions and 5 (12.5%) had involvement of inguinal nodes. Thirty-one patients (77.5%) had complete response after chemoradiation. Only three patients (7.5%) developed chemotherapy-related grade 3 mucositis and myelosuppression. Radiotherapy-related toxicity included grade III cystitis in one patient and grade III proctitis in three patients. Three patients had post-treatment anal stenosis requiring repeated dilatation and two had chronic non-healing ulcers at the anal verge. Nine patients had failure of chemoradiation or disease recurrence; of these, only 5 could undergo salvage surgery. After a median follow up of 60 months, overall survival, disease-free survival and colostomy-free survival were 80%, 77.5% and 72.5%, respectively. CONCLUSION: Chemoradiation is effective in the treatment of squamous cell anal cancer and has acceptable toxicity. Surgical salvage may be useful in those with failure of this treatment.

Surgical management of skin cancers: experience from a regional cancer centre in North India.

AIMS: To review the disease profile and treatment outcome of patients with primary skin malignancies treated at a regional cancer centre. SETTINGS AND DESIGN: Surgical oncology unit of a tertiary care regional cancer centre. Evaluation of treatment outcome of patients with skin cancer from Surgical Oncology database was done. MATERIALS AND METHODS: Retrospective analysis of records of 77 patients with skin cancers treated between 1995 and 2002 was conducted. Profile of patients with skin cancer, surgical details including the management of primary tumour, regional lymph nodes and reconstructive procedures performed and survivals were analysed. STATISTICAL ANALYSIS: All computations were done using the Statistical Package for Social Sciences (SPSS-9). Descriptive statistics were calculated in a standard fashion and survival analysis was performed using Kaplan-Meier method. RESULTS: Skin cancers constituted 2.4% (77/3154) of patients with cancer treated in the surgical oncology department. Squamous cell carcinoma (SCC) was the most common histological type (55.8%) followed by melanoma (26.1%) and basal cell carcinoma (BCC, 18.1%). Forty one percent of patients had undergone some form of intervention elsewhere before being referred. Reconstruction was required in 55.8% patients with large postresection defects. Regional lymph nodal dissection was required in 32.4% of total patients. Five-year median disease-free survival for the entire study population was 75%. CONCLUSIONS: Skin cancers constitute a small but significant proportion of patients with cancer. Unlike in the Western countries, SCC is the commonest histologic variety. Primary level inadequate intervention is very common. Optimal results can be obtained with radical surgery and optimal surgical margins along with a reconstructive procedure when needed.

Patient profile and treatment outcome of rectal cancer patients treated with multimodality therapy at a regional cancer center.

BACKGROUND: Incidence of rectal cancer has wide geographical variation. Disease pattern in developing countries is different from developed countries as majority of the patients present in advanced stage because of delayed referral and lack of uniform treatment practices. AIMS: Present study describes the patient profile and treatment results from a tertiary care cancer center in India. SETTING AND DESIGN: Tertiary care Regional cancer center. Retrospective analysis 89 patients with rectal adenocarcinoma treated between 1995 and 2002 were analyzed. METHODS: Patients with adenocarcinoma rectum were evaluated in a G.I. Oncology clinic and were treated using multimodality protocols involving surgery, radiotherapy and adjuvant chemotherapy. STATISTICAL ANALYSIS: A descriptive analysis of patient and disease profile, treatment patterns and out come was performed. Survival analysis was performed using Kaplan-Meier method. RESULTS: Mean age of the patients was 45.4 years and majority of them had tumor in lower third of rectum with evidence of extrarectal spread. Seventy five percent of the patients underwent curative resection with abdominoperineal resection being the commonest procedure. Forty seven percent of patients were given short course preoperative radiotherapy and the remaining received postoperative radiotherapy. Sixty four percent of patients could complete planned adjuvant chemotherapy. Operative mortality was 2% and 23% had morbidity. Local recurrence rate was 8.9%. 5-year disease free and overall survival was 54% and 58% respectively. CONCLUSION: Majority of rectal cancer patients present with locally advanced and low rectal growths leading to low sphincter salvage rates. Despite the advanced stage of presentation optimal oncologic results can be obtained by using a good surgical techniques in combination with adjuvant radiotherapy and chemotherapy. Short course preoperative radiotherapy seems to be more feasible in Indian context. Timely referral and uniform treatment guidelines throughout the country are needed for optimal management of rectal cancer in India.

A new technique of postoperative pancreatico gastric enterocutaneous fistula repair using de-tubularised afferent loop.

Postoperative fistula involving upper gastrointestinal tract surgery is a rare but troublesome occurrence. Reoperation on these patients is challenging and needs to be individualised. Various methods are described including primary closure, bypass procedures, serosal patch repair and Roux-en-Y repair. We present a new technique of repair using afferent loop that provided a vascularized bowel segment, which used in an anatomical fashion for closure of fistula.