A clinicopathologic profile of adrenocortical tumors.

OBJECTIVE: To study the clinical, biochemical, hormonal, radiological and histopathological profile of adrenocortical tumors in children; to assess the clinicopathological correlations and note the future outcome. DESIGN: Retrospective and prospective study. SETTING: Hospital based; Endocrine Service of our institution and other institution based services. SUBJECTS: 14 children (Females = 11, Males = 3) with adrenocortical tumor, aged 8 months to 13 years (mean age 5.1 +/- 3.42 years), seen over a period of 9 years. RESULTS: Females predominated (F:M = 3.7:1). Majority (64%) had a mixed picture with cushingoid features and virilization, whereas 36% presented only for virilization. Elevated serum cortisol levels with loss of diurnal variation was noted only in those with mixed clinical presentation. Adrenal androgen elevation was noted in majority of cases as virilization was common to all. CT confirmed the diagnosis of tumor, 7 on either side. Thirteen cases were operated. Histopathologic diagnosis was carcinoma in 7 and adenoma in 6 cases. Three of the seven with carcinoma died within 3 months to 2 years but two of these with small tumours (weight 60-65 g and diameter < 6 cm) were well at 2 and 5 years, while as one of the six with a large adenoma had recurrence and metastasis after three years. CONCLUSION: Female preponderance was marked (4 times), 43% of tumors had occurred by 3 years of age and 64% by 6 years. Neither the hormonal parameters nor the histopathology correlated well with the biological behavior and outcome. Prolonged and vigilant follow up is essential.

Undifferentiated embryonal sarcoma of the liver in childhood.

Primary undifferentiated embryonal of the liver in children is a rare neoplasm with dismal prognosis. Surgery is the treatment of choice. Adjuvant chemotherapy may prove useful in improving the prognosis of these tumours. We report two cases of Primary undifferentiated embryonal sarcoma of the liver.

Primary germ cell tumours of the mediastinum.

Twenty nine cases of primary mediastinal germ cell tumours (MGCT) were seen at the Tata Memorial Hospital over a 16--year period (1974-1989). There were 5 benign MGCT occurring predominantly in females (80%), with these patients having an excellent result after surgery with all patients disease free at an median follow-up of 27 months. Malignant MGCT occurred only in males and demonstrated wide variation in response to treatment depending upon whether the tumour was seminomatous or non-seminomatous. There were 11 Seminomas, 5 Embryonal carcinomas, 5 Endodermal sinus tumours and 3 Teratocarcinomas. The diagnosis was established by surgical exploration or by biopsy of a lymph node or chest wall nodule in 20 patients. Four patients had needle biopsy. Seminomatous MGCT received radiotherapy as their main treatment modality and did well with 75% of the patients alive without disease at an average follow up of 33 months. The non-seminomatous MGCT could be divided into two groups. The mean survival for patients receiving cisplatinum based chemotherapy was 14 months as compared to the group not receiving such therapy where the survival was only 5.3 months. However, because of the advanced disease at presentation even in the group receiving cisplatinum chemotherapy, a long term complete response rate of only 20% could be achieved.

Cervical approach for repair of congenital tracheo-esophageal fistula presenting in an adult.

Congenital tracheo-esophageal fistula without esophageal atresia was diagnosed on barium swallow radiography and esophagoscopy findings in an adult patient presenting with difficult in swallowing. The fistulous opening seen on the anterior wall of the esophagus led to the trachea. On bronchoscopy, the tracheal opening was located posteriorly 5 cm below the vocal cords. Successful repair was undertaken via a left cervical approach. The anatomical and developmental basis for the preferred approach is discussed with a brief literature review on the subject.

Choroidal metastasis from primary adenocarcinoma of the esophagus--a case report.

Primary adenocarcinoma of the esophagus is uncommon and the incidence in the middle third is rare, accounting for about 0.7 percent to 1.5 percent. Metastasis of carcinoma to the eye is a rare occurrence. We report here a case of primary adenocarcinoma of the middle third of the esophagus with choroidal metastasis.

Indigenous silicone rubber endoprosthesis for malignant esophago-pulmonary fistula.

Endoscopic placement of an esophageal endoprosthesis is the most rational therapy for relieving the distress of malignant esophago-pulmonary fistula. The commercial prostheses are very expensive for widespread use in India. We have indigenously prepared silicone rubber endoprostheses. The wall of the prosthesis was hardened in a graded manner until the desired resistance to compression with flexibility is achieved. This prosthesis was placed successfully in five patients with malignant esophago-pulmonary fistula. Dysphagia and aspiration were relieved in all the patients. One patient had delayed esophageal perforation and died of massive bleeding 3 weeks after the placement of prosthesis. The indigenous endoprosthesis is cost-effective and safe.

Epiphrenic diverticulum of the esophagus.

Esophageal diverticula are rarely found at the terminal portion, where they are called supradiaphragmatic or epiphrenic diverticula and occur in association with motility disorders of the terminal esophagus. We present here two cases of epiphrenic esophageal diverticulum, one of which was treated surgically.

Giant gall bladder diverticulum.

Diverticula of the gall bladder are very rare. We present here a patient with a giant diverticulum of the gall bladder with features of cholecystitis and cholelithiasis.