Leiomyosarcoma of the esophagus.

Leiomyosarcomas of the esophagus are rare, malignant, smooth-muscle tumors. The presenting symptoms are indistinguishable from other esophageal neoplasms, though the history may be longer due to the slow growth of these tumors. Barium studies may show large intramural masses with ulceration or tracking, expansile intraluminal masses or areas of luminal narrowing. Endoscopic biopsies may give a high false negative rate especially in cases where the mucosa is intact. The treatment of choice is surgical excision. Synchronous and metachronous metastases do not preclude surgery, provided the metastases are also resectable. Prognosis is better than in patients with squamous esophageal cancer. The role of adjuvant radiotherapy and chemotherapy is controversial. We report a 40-year-old man who presented to us with dysphagia and was found to have a leiomyosarcoma of the esophagus. He was treated successfully with esophagectomy and is disease-free after 7 years. We review the literature on esophageal leiomyosarcomas and their management.

Giant pericardial lipoma: unusual cause of intrathoracic mass.

INTRODUCTION: Pericardial lipomas are rare clinical findings. We describe a case of pericardial/epicardial lipoma seen recently at our hospital. CLINICAL PICTURE: A 68-year-old lady presented with a 1-year history of dull aching upper abdominal pain. A chest X-ray revealed a large mass lesion in the right hemithorax. Computed tomography (CT) scan of the chest defined the nature of the lesion. CT-guided fine needle aspiration cytology showed mature fat cells. TREATMENT: The large lipoma (19 x 10 x 4 cm, 1500 g) was completely excised along with a block of the parietal pericardium; through standard right posterolateral thoracotomy. OUTCOME: The patient had an uneventful postoperative recovery. She is currently asymptomatic and has no evidence of recurrence at 18 months follow-up. CONCLUSION: Lipomas in the thoracic cavity are rare and pericardial lipomas even rarer. These are best identified on imaging studies including a CT scan. Complete surgical excision of the lipoma is the only mode of treatment and adequate surgery the only way to prevent future recurrences.

Role of adhesion molecules in recruitment of Vdelta1 T cells from the peripheral blood to the tumor tissue of esophageal cancer patients.

The mechanism responsible for tissue specific localization of gammadelta T cell subsets is not well understood. In order to explain the sequestration of specific gammadelta T cell subsets in the peripheral blood and tumor tissue of patients with esophageal cancer, we examined the function and expression of adhesion molecules on these cells. A hierarchy in the expression of adhesion molecules was observed. In vitro activated gammadelta T cells showed dominant expression of LFA-1 (CD11a), VLA-alpha4 (CD49d), intermediate expression of VLA-alpha5 (CD49e) and L-selectin (CD62L), but low expression of CD44v6 and alphaEbeta7 (CD103). It was observed that the gammadelta T cells use LFA-1, L-selectin and CD44v6 to bind to squamous cell carcinoma (SCC) cells, whereas they adhere to fibroblast cells using LFA-1, VLA-alpha4 and VLA-alpha5. Vdelta1 T cell subsets from the peripheral blood gammadelta T cells utilize a larger array of adhesion molecules, namely LFA-1, VLA-alpha4, VLA-alpha5, L-selectin and alphaEbeta7, to bind to SCC cells compared to the restricted usage of LFA-1, L-selectin and CD44v6 by the Vdelta2 T cells. Flow cytometric analysis of tumor infiltrating lymphocytes from the esophageal tumors confirmed the selective accumulation of Vdelta1+ gammadelta T cells in the tumor compartment. It thus appears that adhesion molecules expressed on these lymphocytes play an important role in the recruitment and retention of Vdelta1 T cells in the tumor milieu.

Inflammatory pseudotumor of the esophagus.

Inflammatory pseudotumors of the esophagus are uncommon. They pose diagnostic and therapeutic dilemmas, especially when located in the cervical esophagus. History and physical examination are rarely contributory. Routine radiologic investigations including barium swallow and computed tomography only raise the suspicion of a benign esophageal neoplasm. Esophagoscopy and biopsy do not provide a definite diagnosis, as these 'tumors' are frequently submucosal, unless they enlarge sufficiently to cause mucosal ulcerations. Endoscopic ultrasonography may accurately localize the tumor but is not diagnostic. Conservative surgical resection or debulking would be both diagnostic and therapeutic. Steroids, cyclophosphamide and low-dose radiotherapy may at best be considered second-line therapy. One such case of inflammatory pseudotumor of the cervical esophagus is presented and the relevant literature is reviewed. Our patient could not be diagnosed on preoperative investigations and required an esophagotomy with frozen section. We debulked the mass and the patient is asymptomatic 6 months after surgery.

gammadelta T cells lyse autologous and allogenic oesophageal tumours: involvement of heat-shock proteins in the tumour cell lysis.

T cells expressing gammadelta receptors were isolated from the peripheral blood of oesophageal cancer patients and analysed for their potential to lyse tumour targets. Immunophenotyping by flow cytometry showed that the dominant population of gammadelta T cells expressed the Vgamma9 and the Vdelta2 T cell receptor, and a minor population expressed the Vdelta1 receptor. Cytotoxicity assays revealed that activated gammadelta T cells lysed Daudi Burkitt's lymphoma and K562 cells. Lysis of autologous oesophageal tumours was higher than of allogenic tumours. Anti-hsp60 and anti-hsp70 mAb significantly inhibited the cytotoxicity of gammadelta T cells to both autologous and allogenic oesophageal tumours. Surface expression of hsp60 and hsp70 on oesophageal tumours and Daudi cells was demonstrated by flow cytometry. In conclusion, gammadelta T cells isolated from the peripheral blood of oesophageal cancer patients have the ability of kill oesophageal tumour cells. The lysis of tumour targets by the gammadelta T cells is brought about via recognition of heat-shock proteins expressed on the surface of tumour cells. gammadelta T cells isolated from the peripheral blood may have applications in adoptive immunotherapy of oesophageal cancer.

Metastatic ovarian neuroblastoma: a case report.

Ovarian metastasis of childhood tumors is rare despite their aggressive nature. The childhood tumor that spreads to the ovary most frequently is the neuroblastoma. The clinical features and frequent bilaterality of ovarian metastatic tumors are helpful diagnostic features in many cases, but when the ovarian tumor is the presenting manifestation of the disease, differentiation from various primary ovarian tumors may be difficult. In this paper, a rare case of metastatic ovarian neuroblastoma is reported.

Endoscopic placement of indigenous plastic esophageal endoprostheses--does it still have a role in the era of expandable metallic stents? A prospective Indian study in 265 consecutive patients.

BACKGROUND AND STUDY AIMS: Esophageal endoprosthesis placement is an established method of palliating inoperable esophageal malignancy. However, the prosthesis choice varies, with expandable metal stents recently gaining popularity. We present our experience of using an indigenously developed plastic prosthesis in 265 patients prospectively in the period April 1992 to May 1996. PATIENTS AND METHODS: An indigenous endoprosthesis made of a medical grade, nontoxic, radiopaque plastic material was placed successfully in 259 patients after serial dilatation of the malignant stricture. Patients were followed up once every month for at least 6 months and also in between if they developed any significant symptoms. The results were analyzed prospectively with special emphasis on the cost of the therapy, technical success of placement, improvement of swallowing and occurrence of complications. RESULTS: The technical success of placement was 97.7% (259/265 patients). The mean dysphagia score improved from 3.2 to 1.2; 212 patients (81.8%) could swallow semisolids whereas 47 patients (18.2%) could swallow liquids. Though 75 patients (28.3%) had an associated tracheoesophageal fistula and 29.8% had received prior radiotherapy/chemotherapy, immediate complications like perforation, respiratory distress or severe hemorrhage were encountered in only 4.3% of patients. Late complications occurred in 12.7% and 32.8% of the patients complained of mild post-procedure pain in the chest. The overall procedure-related mortality was 3.9%. The average cost of the prosthesis was only US$ 15 per patient. CONCLUSIONS: Placement of a plastic prosthesis is still a very effective and safe method for relief of malignancy-induced dysphagia. The associated complications can be significantly reduced by modifying the prosthesis material/design and adhering to a careful technique. The extremely low cost of the prosthesis and its safety profile makes this treatment highly cost-effective and widely applicable in developing countries such as India.

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.

Intrathoracic anastomosis after oesophageal resection for cancer.

Cervical anastomosis has been advocated to avoid the pulmonary complications and life-threatening anastomotic disruptions following intrathoracic oesophagogastric anastomosis. This is a retrospective review of 111 oesophageal resections followed by an intrathoracic anastomosis. These resections were performed between September 1993 and August 1994 within a residency training program. The left thoracoabdominal approach was used for distal tumours and the Ivor Lewis technique for more proximal tumours. Squamous cell carcinoma accounted for 72% patients (n = 80), adenocarcinoma for 25% (n = 28), and others for 2.7% patients (n = 3). Of the patients, 69% had pathologic Stage III tumours. Operative mortality rate was 1.8% (two patients). Perioperative complications occurred in 39 patients, including anastomotic leak in 10 patients and myocardial infarction in 2 patients. In the absence of a leak, there were no major pulmonary complications requiring intensive care or ventilatory support. Of those patients with anastomotic disruption, 80% were salvaged by early clinical diagnosis and appropriate treatment. We conclude that transthoracic oesophagectomy with an intrathoracic anastomosis is a safe procedure that can be performed with low mortality and acceptable morbidity.

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.

Dilation of esophageal strictures induced by radiation therapy for cancer of the esophagus.

During a 2-year period, 103 consecutive patients undergoing dilation of esophageal strictures induced by radiation therapy for cancer of the esophagus were prospectively studied. The length of the strictures ranged from 0.5 to 13.5 cm (median, 5 cm) and the luminal diameter from 1 to 11 mm (median, 6 mm). Patients were referred for dilation from 2 weeks to 5 years (median, 2 months) after completion of radiation therapy. The guide wire was placed using fluoroscopy in 21 patients, endoscopy in 61, and a combination of endoscopy and fluoroscopy in 21. At least one dilator larger than the stricture could be passed in 101 (98%) patients. Five strictures were dilated to 16 mm, 29 to 15 mm, 28 to 14 mm, 16 to 12.8 mm, and 23 to 12 mm or less during the initial procedure. Development of complications and severe resistance were the limiting factors for optimal dilation. Relief of dysphagia was adequate in 66% of patients. The duration of dysphagia relief was 3 to 84 weeks (median, 16 weeks). Complications included persistent pain in 7 patients, unexplained fever in 2, perforation in 2, and delayed tracheo-esophageal fistula in 1. Two patients died of treatment-related complications. Repeated dilation was required in 32 of the 75 patients on long-term follow-up. We conclude that adequate palliation of dysphagia can be achieved by dilation in two-thirds of patients with radiation therapy-induced strictures of the esophagus. Dilation of these strictures is relatively simple and safe if performed with care.

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.

Prognosis of operable squamous cell carcinoma of the esophagus. Relationship with clinicopathologic features and DNA ploidy.

BACKGROUND: Reports on the influence of various prognostic factors in carcinoma of the esophagus are conflicting. The prognostic value of a set of clinicopathologic factors and DNA ploidy were examined in 74 patients with surgically resected squamous cell carcinoma of the lower and middle third of the esophagus. METHODS: All patients had surgery performed in a single thoracic surgical unit at the Tata Memorial Hospital between January, 1984 and December, 1987. The clinicopathologic factors studied were (1) gross residual disease at operation; (2) morphology of the tumor; (3) depth of microscopic invasion; (4) lymph node involvement; (5) histologic grade; (6) vascular and lymphatic embolism; and (7) sex. DNA ploidy and S-phase fraction (SpF) were determined by flow cytometry on archival tissues extracted from paraffin blocks. Ploidy status could be determined successfully in all 74 tumors, whereas SpF could be assessed only in 25. RESULTS: Of the various prognostic factors examined with the Cox stepwise regression model, residual disease (P = 0.000), depth of invasion (P = 0.047), and lymph node status (P = 0.077) were found to be correlated with overall survival. CONCLUSIONS: DNA ploidy was not related to prognosis. The overall survival of this group of patients at 36 months was 28%, and median survival was 18 months.

Choroidal metastasis from primary adenocarcinoma of the esophagus.

Primary adenocarcinoma of the esophagus is uncommon and the incidence in the middle third of the esophagus is rare, accounting for about 0.7%-1.5% of cases. 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.

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.

Long-term right atrial catheters in patients with malignancies: an Indian experience.

Ninety-eight single lumen and two double lumen silicone right atrial Hickman/Broviac catheters inserted in 91 patients with various malignancies over a period of 36 months were prospectively studied. The average duration of use for all Hickman/Broviac catheters was 7,460 days with an average of 74.6 days. A total of 41 episodes of catheter-related infections were documented in all patients (0.5 per 100 access days). Catheter related bacteremia was the most frequent type of infection (31 out of 41, 75%). Majority of the bacteremias could be controlled with routine antimicrobial therapy. Exit site infections were seen in 5 Hickman/Broviac catheters. Two catheters had to be removed because of catheter tunnel infections. Gram negative bacteremic infections were the most common, with Pseudomonas aeruginosa being the major pathogen. Silicone rubber Hickman/Broviac catheters proved to be convenient, reliable, and easily manageable devices in our experience.

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.