LETTERS TO THE EDITOR

 

Synovial two-phase sarcoma in third portion duodenal: clinical case and review of the literature

Sarcoma sinovial bifásico en tercera porción duodenal: caso clínico y revisión de la literatura

 

 

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Key words: Synovial sarcoma. Duodenum neoplasm. Gastrointestinal tumor.

Palabras clave: Sarcoma sinovial. Neoplasia duodenal. Tumor gastrointestinal.

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Dear Editor,

The synovial gastrointestinal sarcoma is an extremely infrequent clinical entity that is usually diagnosed in esophagus distal and gastroesophagical union. We publish the first case of synovial two-phase sarcoma with location duodenal described in the world literature, with a typical inmunohistochemical character.

 

Case report

It is a 70-year-old male who enters of urgencies for clinical suggestive of acute pancreatitis. As precedents of interest the patient is a smoker of approximately 20 cigarettes a day, drinker of approximately 80 g of ethanol a day, suffers COPD in treatment with inhalers, ulcus peptic without symptomatology, biliary lithiasis and senile osteoporosis. In addition, already he had presented episodes of pancreatitis previous solved by conservative treatment.

During his revenue, he presents clinic of debility, anorexia, loss of weight, abdominal diffuse pain, jaundice and coluria persistent. In the blood analyses he presented 18,850 leukocytes (71% neutrophils), GGT of 512 U/ml, alkaline phosphatase of 818 U/ml, platelets of 763.000, bilirubin mg/dl (direct of 3.18 mg/dl) and a Ca 19.9 of 49 U/ml. A CT was realized, in which we appreciates acute nectrotizing pancreatitis Baltasar grade E, by a tenuous captation in pancreatic tail that provokes obstructive jaundice and affectation of vascular structures with splenic infarction (Fig. 1). With the diagnosis of acute nectrotizing pancreatitis, we decided to realize surgical intervention.

After subcostal laparotomy and exhibition of the pancreatic cell, appears a duodenal tumor located in third portion that is extirpated; in addition, there is carried out a cleanliness of the pancreatic cell and cholecystectomy. Hospitable discharge is decided to fourteen days of the intervention.

The anatomopathological analysis of the piece demonstrated that it was a synovial two-phase sarcoma high degree of 9 cm, with inmunohistochemical typical character and with the (X; 18) translocation expressed by means of the reclassification of the gene 18q11.2 with presentation in 13 % of the tumour cells, studied by means of FISH (Table I).

 

Discussion

The synovial sarcomas are tumors that habitually are diagnosed in extremities, principally on knees and in relation with tendons and bursas. Recent finds in the molecular structure of the synovial sarcomas, as the translocation t (X; 18), show the presence of this type of tumors in unusual locations, as pleura, lung and heart. The gastrointestinal tract, of which also there are cases published with this type of sarcomas, continues being an exceptional location, being the most of the published cases synovial sarcomas of esophagus and stomach (1,2).

This type of tumors does not present a specific clinic, but derived from the location of its growth. From the anatomopathological we can classify these tumors in two-phase and single-phase depending on the morphologic presentation, already be estromal and epitelial or only estromal.

The differential diagnosis of these injuries must be done by other neoplasm of the gastrointestinal tract, fundamentally GISTs and sarcomas. The definitive diagnosis will realize by histological analysis and inmunochemist of the piece, that will show focal keratinizations and positivity for the membrane antigen endothelial, in addition they can show areas of microcalcifications and the find of the typical mutation, the translocation (X; 18) SYT/SSX2 (1-3).

The treatment applied till now in these tumors has been the surgery; Makhlouf et al. shows a series of 10 synovial sarcomas, none of them in duodenum, which they resected by segmental sections or atypical gastrectomy and obtained a survival at four years after 60% (4).

In this series, there has been described a recidiva of 20% to 28 months, which it happened in those more undifferentiated tumors and of major size.

It seems, according to the scanty published information, that the forecast of the gastrointestinal sarcoma is in relation with the size, the location and the differentiation of the tumor. They will be deeper necessary studies of the patients with this rare pathology for a better knowledge of the prognosis according to treatment (1-4).

 

S. García-Ruiz¹, R. M. Jiménez-Rodríguez¹, P. Alcaide-León², J. M. Álamo-Martínez¹, G. Suárez-Artacho¹ and M. A. Gómez-Bravo¹
Services of ¹General Surgery and ²Radiology. University Hospitals Virgen del Rocío. Sevilla, Spain

 

References

1. Kawai A, Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 1998; 338(3): 153-60.        [ Links ]

2. Schreiber-Facklam H, Bode-Lesniewska B, Frigerio S, Flury R. Primary monophasic synovial sarcoma of the duodenum with SYT/SSX2 type of translocation. Human Pathol 2007; 38(6): 946-9.        [ Links ]

3. Billings SD, Meisner LF, Cummings OW, Tejada E. Synovial sarcoma of the upper digestive tract: a report of two cases with demonstration of the X;18 translocation by fluorescence in situ hybridization. Mod Pathol 2000; 13(1): 68-76.        [ Links ]

4. Makhlouf HR, Ahrens W, Argawal B, Dow N, Marshalleck JJ, Lee EL, et al. Synovial sarcoma of the stomach: a clinicopathologic, inmunohistoquemical and molecular genetic study of 10 cases. Am J Surg Pathol 2008; 32(2): 275-81.        [ Links ]