2320

Five-Year Survival after Transhiatal Resection of Esophageal Carcinoid Tumor with a Lymph Node Metastasis Christian Partensky, M.D., F.A.C.S.,* Jean Alain Chayvialle, M.D.,* Francoise Berger, M.D.,t Jean-Christophe Souquet, M.D.,* and Bruno Moulinier, M.D.*

Background. Carcinoid tumors of the esophagus are rare. Most reported cases have had a poor prognosis. The authors report the case of a 64-year-old man with a 4-cm carcinoid tumor of the lower esophagus.

Methods. Following endoscopic resection of an ar- gyrophilic, nonargentaffin, carcinoid tumor of the lower esophagus, endosonography showed residual tumor, sug- gestive of a metastatic lymph node. The findings were confirmed at transhiatal esophagectomy.

Results. No recurrence has been observed along the current 5-year follow-up.

Conclusions. Lymph node metastasis does not rule out the possibility of prolonged survival in esophageal carcinoids. Endosonography is strongly advisable for pre- operative evaluation. Cancer 1993; 72:2320-2.

Key words: carcinoid, esophagus, esophagectomy.

Carcinoid tumors of the esophagus are very uncom- mon. Ready et al.' reported one case in 1989 and re- viewed six cases from the English literature. Most of them had a very poor prognosis, even when no meta- static lymph node was detected at tumor resection. Therefore, we considered for publication the case of a patient who remains disease-free 5 years after surgery.

Case Report

In October 1986, a 64-year-old man presented with dyspep- sia and episodic dysphagia. Barium meal and esogastroscopy showed a 4-cm polypoid tumor of the lower esophagus, 30 cm down from incisor teeth. Endoscopic resection of the tu-

mor was performed using a diathermic snare. Histologic exam- ination of the resected specimen revealed a carcinoid tumor (Fig. 1) with no security margin. The vascular network was fairly rich. Cells were argyrophilic (Grimelius reaction, Fig. 2) but not argentaffin (Fontana-Masson staining).

From the *Dbpartement des Sp6cialitCs Digestives and the tLa- boratoire d' Anatomie Pathologique, HBpital Edouard Herriot, Lyon, France.

Address for reprints: Christian Partensky, M.D., Service de Chirurgie Digestive, Dbpartement des Sp6cialiti.s Digestives, HBpital Edouard Herriot, 69437, Lyon cedex 03, France.

Accepted for publication May 26, 1993.

Figure 1. Lamina propria and submucosa are infiltrated by the carcinoid tumor, while the mucosa itself is normal in this area. Tumor is solid and consists of small, uniform cells (H & E, original magnification X60).

Carcinoid of the EsophsguslPartensky et al . 2321

At early follow-up, computerized tomography showed no periesophageal spread. The following hormone concentra- tions were within the normal range: somatostatin, less than 7 pg/ml (n < 50); pancreatic polypeptide, 505 pg/ml (n < 1000); adrenocorticotropic hormone, 20 ng/l at 08:OO am (n < 35); and cortisol 393 nmol/l at 08:OO am (n, 250-660). Esopha- geal endosonography showed that part of the tumor had escaped endoscopic resection. It invaded the mucosal layer only, but a satellite lymph node (1.4-cm diameter) was de- tected between the left inferior pulmonary vein and the aorta.

Transhiatal esophagectomy with gastroesophageal anas- tomosis was performed in June 1987. The postoperative course was uneventful, and the patient was discharged from the hospital on day 13.

At histology, the tumor invaded the mucosa and the sub- mucosa, and one of the nine resected lymph nodes was meta- static. Immunostaining for chromogranin A was positive. An- tisera respectively directed against gastrin, somatostatin, pancreatic polypeptide, insulin, glucagon, calcitonin, beta- endorphin, and 17-39 adrenocorticotropic hormone failed to bind to any immunoreactive material. Numerous secretory granules, ranging in size from 100-250 nm, were observed at electron-microscopy (Fig. 3).

Figure 3. Electron microscopy. Secretory granules are observed in tumor cells (arrow). Scale given by bar in upper right corner (0.5 pm) (H & E, original magnification X28,OOO).

The patient has been followed-up since that time an- nually. In July 1992, he was asymptomatic. Clinical examina- tion, esogastroscopy, and thoracoabdominal CT scan showed no evidence of recurrence and no metastasis.

Discussion

The presence of argyrophilic cells in the esophageal mucosa presumably accounts for the development of neuroendocrine carcinomas.2

Among them, oat cell carcinomas resembling bron- chogenic tumors are the most frequent type.3 The histo- logic pattern consists of small, spindle-shaped cells, grouped as solid sheets of anastomosing cords. The presence of argyrophilic cells is fairly frequent. Amy- loid deposits are occasionally o b ~ e r v e d . ~ . ~

True carcinoid tumors are rare. At histology, they consist of fairly to well-diff erentiated argyrophilic

Figure 2. Grimelius staining. Tumor cells are typically argyrophilic (H & E, original magnification X 160).

2322 CANCER October 15, 1993, Volume 7 2 , No. 8

cells: organized as solid nests, or as acinar or tubular structures. Another suggestive feature is the abundance of fibrous stroma.

The present case displayed pathologic, histochemi- cal, and ultrastructural characteristics that were fully consistent with those of a foregut carcinoid tumor, The argentaffin negativity made it distinct from a serotonin- oma. No circulating marker was identified through the tested assays. The immunocytologic study was nega- tive, but immunoreactivity for vasoactive intestinal peptide, which has been reported in another case,7 could not be tested.

The long-term course of the patient, who is alive and disease-free more than 5 years after diagnosis, is at variance with the dismal prognosis of most reported esophageal carcinoids. In a review of 163 patients with tumors classified as neuroendocrine, carcinoid, small cell, oat cell, or anaplastic carcinomas, mean survival after diagnosis was 6.2 months (range, 0-33). Fifty-four patients underwent surgical resection of the tumor; sur- vival in this subgroup ranged from 1-28 months, with a mean value of 6.5 months.8 One patient received adju- vant chemotherapy and was considered to be in com- plete remission 2 years after resection.' This encourag- ing result was not confirmed in another study, where three patients survived for 21,20, and 9 months, respec- tively, despite adjuvant chemotherapy and radiation therapy."

The preoperative assessment of the limited tumor spread, through computerized tomography and endo- sonography, was of upmost value to elect transhiatal esophagectomy, given the location of the tumor in the lower esophagus. The procedure allowed for the re- moval of several satellite lymph nodes, including the metastatic node that had been detected at echoen- doscopy .

The present case illustrates that prolonged survival may result from resection of an esophageal carcinoid, even when it is recognized at a stage of lymph node invasion. Limited potential for distant metastases, or very low growth rates of minute metastatic foci, may be intrinsic features of the present tumor that played a crucial role in the outcome. From a practical standpoint, endosonography is suggested to be a major advance in preoperative selection of the surgical procedure.

References

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2. Tateishi R, Taniguchi H, Wada A, Horai T, Taniguchi K. Argyro- philic cells and melanocytes in esophageal mucosa. Arch Pathol 1974; 98:87-9.

3. McKeown F. Oat cell carcinoma of the esophagus. ] Pathol Bac- teriol 1952; 64:889-91.

4. Tateishi R, Taniguchi K, Horai T, Iwanaga T, Taniguchi H, Ka- buto T, et al. Argyrophil cell carcinoma (apudoma) of the esopha- gus: a histopathologic entity. Virchows Arch [A] 1976; 371:283- 94. Doherty MA, McIntyre M, Arnott SJ. Oat cell carcinoma of esophagus: a report of six British patients with a review of the literature. Int Radiat Oncol Biol Phys 1984; 10:147-52.

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10. Attar BM, Levendlglu H, Rhee H. Small cell carcinoma of the esophagus: report of three cases and review of the literature. Dig Dis Sci 1990; 35:145-52.

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