Esophageal carcinoma originating in a duplication cyst: case report

Carcinoma em cisto esofágico duplicado: relato de caso

14 – CASE REPORT

ESOPHAGEAL CARCINOMA ORIGINATING IN A DUPLICATION CYST. CASE REPORT. ¹

Amadeu P. A. Pimenta²

Maria Leonor David³

Manuela B. Baptista⁴

Elizabete R. Barbosa⁵

Pedro Varzim⁶

PIMENTA, A.P.A.; DAVID, M.L.; BAPTISTA, M.B.; BARBOSA, E.R.; VARZIM, P. - Esophageal carcinoma in a duplication cyst: case report. Acta Cir. Bras, 12(2):213-6, 1997.

SUMMARY: The authors present the case report of a 61-year-old man, admitted with middle third squamous cell esophageal carcinoma. He was submitted to a curative gastroesophageal resection via a medium laparotomy and a right thoracotomy. An intrathoracic esophagogastric anastomosis was performed. The pathological analysis of the surgical specimen revealed a squamous cell carcinoma clearly originating from the epithelial lining of an esophageal duplication cyst. Immunohistochemitry showed p 53 staining of the tumor cells. The patient at 11 month follow up was asymptomatic.

SUBJECT HEADINGS: Esophageal cyst. Esophageal carcinoma. Esophagus

INTRODUCTION

In spite of the clinical silence of some congenital lesions, nowadays, with the new methods of imaging, (CAT, Ecoendoscopy and NMR), not only are these lesions diagnosed in an asymptomatic stage but also, more importantly, surgeons are frequently asked to decide whether or not to operate on these asymptomatic lesions.

Esophageal cysts are rare lesions corresponding to 10 - 20 % of the benign tumors of the esophagus⁷. Although they generally don’t cause symptoms that interfere with leading a normal life, they can break their clinical silence when they inflame, bleed, ulcerate or cause obstruction of the esophagus^1,4,6,11. It is, however, above all, the knowledge that these lesions can become malignant that convinces surgeons to propose surgical treatment of the cyst in the asymptomatic stage.

Although we can find some case reports about malignant transformation of esophageal diverticula^2,9 and also a case of malignant transformation of an esophageal cyst diagnosed by autopsy⁷, to the best of our knowledge this case report of a successfully operated intramural squamous cell carcinoma.

CASE REPORT

A 61-year-old caucasian man was referred to Service Cirurgia 4 of Hospital S. João, Porto, Portugal, in february 1996. The diagnosis on admission was a middle third esophageal squamous cell carcinoma. The patient had noted a gradual weight loss, (5 kg over 7 months), and two months prior to his admission to the Hospital. He sought medical attention upon the development of dysphagia for solid foods, epigastric discomfort and fullness after meals and regurgitation of food. He smoked a packet of cigarettes daily for 38 years and consumed alcoholic beverages, (80 g daily) , for 50 years. He didn’t normally eat smoked or salty foods.

The physical examination was normal. Laboratory studies and chest X- ray were normal. A barium swallow showed an obstructive narrowing lesion in the middle third of esophagus and some dilatation of the esophagus above this stenosis (fig.1). An infiltrative lesion was observed at 30cm from the incisors during esophagoscopy. It occupied 2/3rds of the esophageal lumen and blocked the passage of the fibroscope. A biopsy revealed a squamous cell carcinoma. A CAT showed a concentric, swollen esophageal wall starting at the level of the bronchial arch and continuing downwards for 5cm without apparent invasion of the neighboring structures. On the sixth day in hospital a right thoracotomy and a medium laparotomy were carried out allowing a curative resection of a hard esophageal tumor situated at a level of the bronchial arch. An intrathoracic esophagogastric anastomosis was performed. The patient has done well and at 11 month follow up was asymptomatic.

PATHOLOGICAL FINDINGS

Three biopsy specimens were collected during endoscopy. Histologic examination showed, in two of the specimens, a squamous cell carcinoma occupying the submucosa and muscle layers without involving the mucosa.

The surgical specimen included 9cm of the distal esophagus and cuff of proximal stomach (fig. 2). In the esophagus, a circumpherential lesion, occupying the whole depth of the esophageal wall but not involving the overlying mucosa was observed. The histologic examination showed a squamous cell carcinoma clearly originating from the epithelial lining and the esophageal mucosa was also identified. Metastases were observed in 6 out 13 lymph nodes isolated in the surgical specimen. Immunohistochemitry showed intense nuclear stainig of most tumor cells for p53 (imunotech; Do-1) (fig.4). Four hemorragic papules were visible at the esophageal mucosa in the vicinity of the tumor (fig.2). Histologically they corresponded to hemorragic dissociation of the squamous cells from the epithelial lining of the esophageal mucosa. Immunohistochemistry, using antibodies directed to Herpes virus and Citomegalovirus, and electron microscopy did not reveal the presence of viral particles.

DICUSSION

At about the 5^th week of embryological development the esophagus is a solid body⁴. However, between the 5^th and 8^th week vacuoles develop and coalesce within the esophagus leading to the formation of the esophageal lumen⁴. When the development of the esophagus doesn’t proceed normally, an epithelium-lined pocket may remain in the esophageal wall. This pocket can become a diverticulum or even a completely closed duplication cyst within the esophageal wall, separated from the lumem of the esophagus by a septum of connective tissue covered on both sides by a mucosa⁴. Fewer than 10% of duplication cyst have an opening into the esophagus, thus allowing the discharge of secretions⁹. In our case the lesions looks more like a cyst than a diverticulum, bearing in mind its intra-mural position in the middle part of the esophagus and the absence of esophageal symptoms before the beginning of progressive dysphagia.

The unusualness of carcinomas occurring in esophageal intra-mural cysts is probably explained, firstly, by the rarity of these cysts and, secondly, by the absence of irritative factors in direct contact with the mucosa of the cyst due to the absence of communication between the cyst and the esophageal lumen. The development of malignant carcinoma in a duplication cyst probably contact with alimentary aggressors. However, in this case tobacco and alcohol, the two factors in Western countries most frequently related to the etiopathogeny of squamous cell carcinoma of the esophagus^3,8, were both present. Some others factors potentially involved in carcinogenesis, particularly viral, were found not to be present in our case.

From a clinical point of view, it is important to stress that both the barium swallow and the TAC showed a regular-shaped stenosis, that could be interpreted as a benign lesion. This radiological aspect is explained by the integrity of the esophageal mucosa covering the tumor, as confirmed by the appearance of the surgical specimen.

Although the beginning of dysphagia or some other symptoms in a patient with a cyst can be explained by an ulceration of the cyst, we should always bear in mind that these symptoms can also occur due to a malignant transformation of the cyst. Therefore, a biopsy of greater depth than needs to be carried out in order to achieve an accurate diagnosis.

We observed p53 immunoreactivity in the carcinoma cells but not in the nonneoplastic cells from the epithelial lining of the esophageal cyst or the overlying esophageal mucosa.

The overall percentage of p53 immunoreactivity in esophageal carcinomas is high 74% in a recent report by PARENTI, RUGGE, FRIZZERA, RUOL, NOVENTA, ANCONA e NINFO⁸. Interestingly, when mutation analysis is performed. A high frequency of G-T transversions is reported, which suggests a direct mutagenic effect of cigarette smoke⁵.

In the present case, mutation analysis was not performed and so we cannot exclude the possibility that the carcinogens that act the esophageal lining are also able to reach and act on the epithelium of esophageal cysts. Future studies should address this possibility because, if a link can be made between canceruzation of the cyst lining and the presence of major risk factors for malignant transformation in the esophagus, this would help surgeons to take a more aggressive attitude in asymptomatic patients who accumulate such risk factors.

Nowadays, whit the increasingly high-definition possible with modern methods of imaging, surgeons are frequently asked to decide whether or not to operate on asymptomatic lesions.

CONCLUSIONS

Esophageal duplication cysts are rare, and commonly asymptomatic, lesions. Like other benign diseases of the esophagus, they can inflame, bleed, ulcerate, cause obstruction and, as is clearly shown in this case report, also become malignant.

Even with modern methods of imaging, the diagnosis of malignant transformation of the cyst can be difficult. Therefore, once diagnosed, surgical resection should be the treatment of choice even for asymptomatic and apparently benign cysts.

PIMENTA, A.P.A.; DAVID, M.L.; BAPTISTA B.; BARBOSA, E.R.; VARZIM, P. - Carcinoma em cisto esofágico duplicado: relato de caso. Acta Cir. Bras., 12(3):213-6, 1997.

RESUMO: Apresenta-se um caso de homem com 61 anos de idade, portador de carcinoma do terço médio do esôfago. Foi submetido a ressecção gastro-esofágica radical através de laparotomia mediana toracotomia direita. O exame histopatológico mostrou carcinoma de células escamosas nitidamente originado da camada epitelial de cisti esofágico duplicado. Mostrou-se mediante imunohistoquímica células tumorais coradas para p53. Em 11 meses de evolução o paciente permanece assimtomático.

DESCRITORES: Cisto esofágico. Carcinoma esofágico. Esôfago.

Address reprint request:

Prof. Dr. Amadeu Pimenta

Serviço de Cirurgia 4 - Hospital de S. João, 4200

Porto - Portugal

Tel: [phone] - Fax: [phone]

Accepted for publication on January, 1997.

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1. From the

. From the Cirurgia 4 Unit - Hospital S. João, Porto, Portugal

2. Medical Doctor

3. Medical Doctor - Department of Pathology / Institute of Molecular Pathology (IPATIMUP)

4. Medical Doctor

5. Medical Doctor

6. Medical Doctor - Department of Radiology, Faculdade Medicina do Porto, João, Porto, Portugal

Publication Dates

• Publication in this collection
  08 June 2001
• Date of issue
  Sept 1997

History

• Accepted
  Jan 1997