ABSTRACT
Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma of low to intermediate malignant potential. Incidence is estimated to be 0.8 to 4.5 cases per million persons per year. The tumor is highly aggressive locally and is known to recur. Punch biopsy or excisional biopsy (in case of small tumors) are diagnostic. Computer tomography (CT) and magnetic resonance imaging (MRI) are useful in determining the size and extent of the tumor’s treatment is primarily surgical, with a wide local excision with at 2 cm margin. However, local recurrence after apparently adequate surgical excision is common. Mohs micrographic surgery has been recommended as it enables maximum preservation of tissue. When surgery is insufficient, imatinib mesylate is shown as a safe and effective treatment in dermatofibrosarcoma protuberans (DFSP), especially in cases of locally advanced or metastatic disease. Here, we report a case of a giant fibrosarcoma on the anterior abdominal wall, measuring 27×18×9 cm, which occurred in a recurrent dermatofibrosarcoma protuberans. Diagnosis was done by histopathological examination (HPE) of the previously excised tumor and CT was done. The tumor was excised with a 3 cm margin and extensive reconstruction of the anterior abdominal wall defect was done using synthetic mesh, myocutaneous flaps and split skin grafting. Keeping in view the recurrent nature of our case, large tumor size, DFSP-fibrosarcomatous (FS) transformation and close negative margins in the HPE of the postoperative specimen, the patient was planned for adjuvant radiotherapy.
ABSTRACT
Background: Despite the surgical treatment, sophisticated intensive care units, latest generation antibiotics and a better understanding of pathophysiology, the morbidity and mortality rate of perforation peritonitis are still high. Patients are usually managed by subjective decision of surgeon based on which mortality is very high.Methods: This was a double-blind observational study conducted over a period of 18 months on 50 patients with small bowel perforations. Based on the acute physiology and chronic health evaluation (APACHE) II score at presentation, patients were triaged into 3 groups: group 1 (score ≤10), group 2 (score 11 to 20) and group 3 (score >20). Study population was managed by the subjective decision of the operating surgeon who was blinded off the APACHE II score of patients. Hence removing the possibility of bias and observing a correlation between surgical outcome and APACHE II score of the patient.Results: Patients with higher APACHE II score (>10) were more likely to undergo exteriorization of bowel. Length of hospital stay was also found to be increased with an increase in score. APACHE II score of 10 was found to predict mortality with significant difference between 2 groups. Below this score the mortality was 0% and above this score the mortality rate rose to 31.25%.Conclusions: APACHE II can be used as a reliable and uniform scoring system as its assessment at presentation in patients of small bowel perforations provides an insight to their surgical management as well as predicting overall outcome.