Abrikossoff's tumor--a rare differential diagnosis in neoplastic lesions of the esophagus

We report the case of a 49-year-old male patient who was referred to our department in February 1998. Due to his complaints of dysphagia and retrosternal pain an upper gastrointestinal endoscopy was performed showing a polypoid lesion of the lower esophagus. This lesion presenting with a wide base and indurated surface was removed by endoscopic snare and forceps biopsies. Histologic and immunohistochemical analyses revealed this lesion to be a benign granular-cell tumor (Abrikossoff's tumor) which is considered to be of neuroectodermal origin. Abrikossoff's tumor is a rare differential diagnosis of polypoid lesions of the esophagus.     

Granular cell tumor of the esophagus

A case of granular cell tumor of the esophagus in a 50-year-old man is reported. Gastrointestinal endoscopy revealed a round, sessile, non-ulcerated white-yellow elevated tumor at the lower third of the esophagus. Biopsy revealed a granular cell tumor. Immunohistochemical staining demonstrated that granules in the cytoplasm of tumor cells were positive for S-100 protein and negative for carcinoembryonic antigen. An electron microscopic study revealed that tumor cells were closely packed in clusters, surrounded by basal lamina and collagen fibers. Most cells contained dark cytoplasm filled with electron-dense granules. These granules resembled lysosomes and phagosomes. In a few cells with clear cytoplasm, some mitochondria and poorly developed endoplasmic reticulums were seen. Fibrillar internal materials, myelin-like figures and a premature angulate body were observed in the clear cytoplasm. The lesion has remained unchanged in gross appearance and in size for twenty-three months without any treatment.     

Granular cell tumors of the esophagus

An unusual benign multicentric esophageal granular cell tumor (granular cell myoblastoma) associated with 16 other similar tumors in the skin, vulva, breast, and tongue of 1 patient is described. There was a family history of granular cell tumors in the patient's mother; this has not been previously described. The pathologic findings and controversial histogenesis of granular cell tumors are discussed in an effort to delete the erroneous term "myoblastoma" from the radiologist's vocabulary. Granular cell tumors of the esophagus are also specifically reviewed.     

Granular cell myoblastoma of the esophagus

Granular cell myoblastoma, a rare, but generally benign tumor, is being encountered with increasing frequency in the digestive tract. Seventy-two cases have been described in the esophagus; we report two additional cases. A follow-up, available in 13 patients (including ours), reveals a low likelihood of malignant transformation or recurrence after surgical removal. Guidelines for the management of these tumors are presented.     

Granular cell tumors of the esophagus.

Three cases of granular cell tumor of the esophagus are added to the 17 previously reported in the literature. These tumors, thought to be of neural origin, are difficult to diagnose preoperatively. The diagnosis should be considered in adult females presenting with an intramural mass of the proximal or distal third of the esophagus. Symptoms of dysphagia and substernal discomfort are likely to occur with lesions greater than one centimeter in diameter. Preoperative biopsy is not advised as a mistaken diagnosis of squamous cell carcinoma can result.     

Granular cell tumors of the esophagus. 5 cases

The authors report 5 new cases (3 women, 2 men) of granular cell tumours of the oesophagus. Two patients were treated by endoscopic resection with a diathermy loop, two patients underwent surgical ablation and the other tumour was discovered at autopsy. There were no complications related to treatment and there have been no recurrences to date. Endoscopic resection of these tumours would seem to be the treatment of choice when their size is less than 30 mm.     

Granular cell tumor--a unique neoplasm of the internal anal sphincter: report of a case

Granular cell tumors are rare, invariably benign, and often solitary tumors, which infrequently involve the gastrointestinal tract. We report the unique presentation of a granular cell tumor of the internal anal sphincter in a 75 year-old female. The tumor was detected during investigation of new rectal bleeding and was excised using a transanal approach and sphincter repair. At five-year follow-up the patient reported normal continence to stool and flatus and demonstrated no evidence of tumor recurrence. Immunohistochemical studies cite the Schwann neural cell as the origin of the granular cell tumor. Cytoplasmic features include acidophilic, p-aminosalicylic acid-positive, diastaseresistant granules. Granular cell tumors may be located anywhere in the body, but anorectal involvement is rare. In our own search of the world literature, no other cases were reported specifically to involve the anal sphincter. Granular cell tumors are usually detected incidentally but may be symptomatic, especially when the anorectal region is involved. Symptoms include perianal discomfort and bleeding. Adequate local excision is effective for both diagnosis and treatment of anorectal granular cell tumors. Careful follow-up should be performed after excision because of the risk of recurrence.     

Granular cell tumour of the oesophagus: a rare cause of dysphagia with differential diagnosis of oesophageal neoplastic lesions

Granular cell tumour is a relatively uncommon, typically benign neoplasm of soft tissue. The macroscopic appearance of oesophageal granular cell tumour is a polypoid lesion, which is often asymptomatic and can be found incidentally, but, in some cases, is symptomatic and requires a correct differential diagnosis with malignant neoplasms of the oesophagus. We describe the case of a 28-year-old female who came to our attention due to a six-month history of heartburn and dysphagia. Oesophagogastroduodenoscopy showed the presence of a polypoid lesion 2 cm above the gastro-oesophageal junction. The overlying mucosa was normal and the lesion seemed to be an isolated submucosal nodule with a “submucosal pill” appearance. It was excised completely using a standard diathermic snare, and diagnosis of oesophageal granular cell tumour was made by histological and immunohistochemical staining. The patient's symptoms disappeared immediately after removal of the nodule by endoscopic polypectomy, and no macroscopic or microscopic recurrence of granular cell tumour was noted during follow-up. Likewise, the patient was symptom-free during follow-up. This case shows that endoscopy is very effective, not only in the diagnosis, but also in the treatment of oesophageal lesions which require careful differential diagnosis.     

A rare primary small cell cancer of the esophagus

The parvicellular carcinomas have their matrix in the neurosecretory (enterochromaffin) cells of Kultschitzky's type of the APUD-system. While in about 20% of the patients with a bronchial carcinoma a parvicellular bronchial carcinoma appears, extrapulmonary parvicellular organ carcinomas are very rarely observed. Up to 1984 altogether 72 patients with a parvicellular carcinoma of the oesophagus were world-wide registered. It is reported on an own observation of a female patient with a parvicellular carcinoma of the oesophagus and it is referred to the appearance of extrapulmonary parvicellular carcinomas. According to the present state of knowledge the extrapulmonary parvicellular carcinomas should be treated according to the therapy concepts of parvicellular bronchial carcinomas.     

Granular cell tumor of the esophagus: description of an infrequent benign tumor

Granular cell tumors (GCT) are infrequent tumors first described by Abrikossoff in 1926. Gastrointestinal involvement occurs in about 6% of GCT, the esophagus being the most frequent location. These tumors are usually benign and asymptomatic and are usually found incidentally when an upper gastrointestinal endoscopy is carried out for another reason, showing an isolated polyp or sessile submucosal nodule, covered by intact yellowish mucosa and with firm consistency. Endoscopic ultrasonography has significantly improved the diagnosis of these lesions. Nowadays endoscopic mucosectomy is the treatment of choice of esophageal GCT with a low frequency of complications. Histologic analysis of the surgical specimen shows specific characteristics such as positivity for S-100 protein. We present two new cases of esophageal GCT that were diagnosed recently and discuss the most relevant features of this infrequent disease.     

Granular cell tumor of the esophagus: endoscopic ultrasonographic demonstration and endoscopic removal

A 35-yr-old Japanese man with a granular cell tumor of the esophagus that was removed by endoscopic polypectomy is presented. Radiography and endoscopy showed a 20 x 12 mm sessile protrusion in the distal esophagus. Endoscopic ultrasonography demonstrated the hypoechoic mass in the submucosa without continuity to the muscularis propria. The lesion was successfully treated by endoscopic polypectomy without complications. The cross-sections of the resected specimen were quite in agreement with the ultrasonographic findings. Endoscopic ultrasonography is valuable to assess the exact location and extent of the tumor, and to determine the indication for endoscopic polypectomy.     

Gastric stromal tumor--a rare cause of an upper gastrointestinal bleeding

Gastrointestinal stromal tumors (GISTs) are rare neoplasms arising from connective tissue elements of the gastrointestinal wall. They show a great heterogeneity with respect to their histogenetic, morphologic and prognostic characteristics. GISTs are known with myoid, neural or mixed features of differentiation. Clinical findings are gastrointestinal bleeding, abdominal pain and weight loss. We report on the case of a 50-year-old male patient who presented with melena and acute anemia (hemoglobin 10.5 g/dl). Esophagogastroduodenoscopy revealed a broad-based, centrally ulcerated polypoid formation of 3 cm in the gastric corpus as the cause of the upper gastrointestinal bleeding. Multiple endoscopic biopsies were negative for neoplastic changes. Because of no tendency of healing after triple eradication therapy of Helicobacter pylori and following proton pump inhibitor medication, the patient underwent distal gastrectomy with gastrojejunostomy. GIST of combined smooth muscle and neural type was diagnosed by histological and immunohistochemical examination. The features with increased mitotic activity and cellularity were those of a borderline stromal tumor. 6 months after surgery the patient is well with no signs of residual malignancy. This case demonstrates that rare stromal neoplasms have to be taken into account in the differential diagnosis of gastrointestinal tumors even if endoscopic biopsies are negative for neoplastic changes. Because of the uncertain biological behavior of the GISTs an early surgical intervention is recommended.     

Granular cell tumor of the esophagus: Natural history,diagnosis, and therapy

Five cases of granular cell tumor of the esophagus are reported. In four cases, the tumor was an asymptomatic, incidental finding. In one case, a larger granular cell tumor presented with dysphagia and required local surgical excision. Long-term follow-up in three cases revealed no evidence of tumor progression. Esophageal granular cell tumors are benign lesions which can frequently be diagnosed by endoscopic biopsy. Asymptomatic, smaller lesions require observation only. Larger, symptomatic lesions can be treated with local surgical excision.     

Granular cell tumor of the esophagus: report of five cases and review of the literature

Granular cell tumor (GCT) is an uncommon esophageal neoplasm. It commonly presents as a nonspecific painless mass. The purpose of this study is to describe our experience with 5 patients newly diagnosed with GCT at VA Medical Center in Memphis, Tennessee from February 2001 to June 2005. Clinical manifestation, endoscopic appearance, histology, different modalities of treatment and prognosis are discussed. Based on this experience, we conclude that GCTs are relatively rare in occurrence. They usually present as a firm to hard submucosal nodule on esophagogastroduodenoscopy. Diagnosis can be made by endoscopic biopsy in most cases. Treatment options include endoscopic surveillance, endoscopic resection, or surgery. The usual course of GCTs is benign.     

Choriocarcinoma - a rare association with squamous cell carcinoma of esophagus.

Extragenital choriocarcinoma involving the gastrointestinal tract is rare. We report a 60-year-old woman with squamous cell carcinoma of esophagus with a choriocarcinomatous focus. She was palliated with chemotherapy and an endoprosthesis.     

A rare pituitary lesion

The majority of the sellar masses are pituitary adenomas. Non-adenomatous hypophyseal lesions are rare and include lymphocytic hypophysitis, idiopathic giant cell hypophysitis and granulomatous hypophysitis. The latter include lesions caused by conditions like tuberculosis, sarcoidosis, syphilis, histiocytosis X and mycotic infections. We report a case of a Sudanese woman who presented with a pituitary mass (presumed preoperatively to be an adenoma) and underwent trans-sphenoidal surgery. The final histopathology showed tuberculous hypophysitis. The patient did not have history of prior exposure to tuberculosis nor any evidence of infection elsewhere in the body. We also briefly review the literature on pituitary tuberculomas.