Intrinsic diseases of the adult esophagus: Benign and malignant tumors

Intrinsic diseases of the adult esophagus: Benign and malignant tumors

Intrinsic Diseases of the Adult Esophagus: Malignant Tumors Harvey M. Goldstein, Jesus E SOPHAGEAL neoplasms are among the least common types of ...

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Intrinsic

Diseases of the Adult Esophagus: Malignant Tumors Harvey

M. Goldstein,

Jesus

E

SOPHAGEAL neoplasms are among the least common types of gastrointestinal tumors. Difficult or painful swallowing are the most common symptoms. Virtually all neoplasms of the esophagus are first discovered on esophagography. Occasionally small tumors are discovered incidentally during a barium study for other reasons. Both benign and malignant tumors occur at any location within the esophagus and have a wide variety of radiologic configurations. BENIGN

TUMORS

Benign tumors of the esophagus occur relatively infrequently, less commonly than malignant tumors. Plachta’ has indicated the relative frequency of benign tumors as follows: leiomyoma and other myomas 5 1%, polyp 25%, cyst 8%, papilloma 3%, fibroma 3%, and hemangioma 2%. The remaining 8% consists of extremely rare tumors, such as myxofibroma, neurofibroma, adenoma, etc. Their incidence in large autopsy series is much less than 1%. There is no pathologic classification of benign esophageal neoplasms that is universally accepted. The tumors may be subdivided into those having intraluminal or intramural origins (Table 1), but many benign tumors have both an intraluminal and intramural component.2 lntraluminal

Polyps are the most common and most important of the intraluminal lesions. Microscopically, they can be divided into the fibrovascular p01yp,~ lipoma,4 and inflammatory fibrous p01yp.~ Some of these polyps may attain a large size and develop a long pedicle, usually attached at the level of the cervical esophagus. Most dramatically, these large masses may occasionally prolapse into the patient’s mouth or obstruct the airway.2 The papilloma is usually small and sessile; it may be multiple (Fig. l).6,7 Its chief importance lies in its resemblance to a small squamous cell carcinoma. Intramural

Leiomyoma accounts for more than half of the benign esophageal tumors. Over half of the Seminws

in Roentgenology,

Vol.

XVI.

No. 3 (July),

1981

Zornoza,

and Theodore

Benign and

Hopens

patients with esophageal leiomyoma are asymptomatic. The rest have either dysphagia or substernal chest pain.’ In profile on esophagography it appears as a smooth, semicircular, or ovoid defect in the barium column. The superior and inferior margins of the tumor make a right or slightly obtuse angle with the wall of the esophagus (Fig. 2). 9.‘o When visualized en face, the leiomyoma has a polypoid appearance and stretches the esophageal folds. Not infrequently, an cxtraesophageal soft tissue component of a leiomyoma may be visualized as it interfaces with the airfilled lung adjacent to the mediastinum. Usually, the radiographic appearance of the tumor is typical; however, endoscopy is helpful to confirm the integrity of the mucosa overlying the tumor. In one small series, the patients were followed over a period of years, and no evidence of gross malignant degeneration occurred.” Most leiomyomas are between 2 and 8 cm in diameter. They are multiple in less than 3% of cases;‘,‘* multiple esophageal leiomyomas may be associated with uterine and vulvar myomas in late childhood.‘3 Esophageal cysts are the next most common benign mass lesions of the esophagus.’ The cysts are either congenital or acquired, the former being more frequent. Congenital cysts are subdivided into several subclasses based pri-.~--____ From the Department of Radiology, Southwesr Texas Methodist Hospital, San Antonio: the Depariment of Diagnostic Radiology, University of Texas System Cancer Center. M. D. Anderson Hospital and Tumor Institute, Houston; and the Department of Diagnostic Radiology, University of Texas Health Science Center, San Antonio, Texas. Harvey M. Goldstein, M.D.: Radiologist, S.W. Texas Methodist Hospital and Clinical Professor of Radiology, University of Texas Health Science Center; Jesus Zornoza, M.D.: Associate Professor of Diagnostic Radio1og.v. University of Texas Systems Cancer Center: Theodore Hopens, M.D.: Assistant Professor of Radiology, University of Texas Health Science Center. Reprint requests should be addressed to Jesus Zornoza. M.D., 6723 Bertner. Houston, Texas 77030. 0 1981 by Crune & Stratton, Inc. 0037- r98X/81/1603-0005$02.00/0 183

GOLDSTEIN,

184

Table

1. Benign

Tumors

of the

ZORNOZA.

AND

HOPENS

Esoohaous

lntraluminal Polyp Fibrovascular Lipomatous Inflammatory Papilloma Others Intramural Leiomyoma Cyst Hemangioma Granular

cell tumor

Fig. 2. Leiomyoma. Double contrast esophagography shows a smooth mass arising from the anterior wall of the midthoracic esophagus. The right angles with the esophageal wall indicate a submucosal origin.

Fig. 1. Squamous cell Double contrast esophagram lar mucosal nodules along

papillomatosis of the esophagus. demonstrates multiple irreguthe entire thoracic esophagus.

marily on the relative content of foregut and respiratory tract elements. The esophageal cyst usually presents as an intramural or extrinsic mass in the lower esophagus (Fig. 3).14 When multiple, they may be caused by the entity of esophagitis cystica. Esophageal hemangioma may be an isolated lesion or associated with Osler-Weber-Rendu syndrome. In either situation, it is benign and usually intramural. It may reach considerable size and give rise to gastrointestinal bleeding.” Granular cell tumor is an intramural lesion that is thought to be of neural origin.16

ESOPHAGEAL

185

TUMORS

The gastroesophageal inflammatory polyp associated with esophagitis or a gastric fold prolapsed into the distal esophagusmay have the appearance of an intraluminal polypoid esophageal tumor.” Usually there is continuity of these intraluminal defects with gastric folds at the cardia. MALIGNANT

TUMORS

Virtually any narrowing, mass,or ulcer in the esophagus must be considered suspect for an esophagealcancer. The vast majority of esophageal cancers cause luminal narrowing, and the

Fig. 3. ing defect esophagus.

Differential

Esophageal involving the

cyst. Smooth, posterior wall

well-marginated of the midthird

fillof the

Diagnosis

The differential diagnosis of benign esophageal tumors includes a number of diverse entities. An intramural lesion is most often confused with an extrinsic anatomic structure or mass, particularly if the latter is adherent to the esophagus. On occasion, a polypoid malignant esophagealtumor may stimulate a benign intraluminal or intramural tumor. Irregular contour strongly favors malignant tumor. A benign intraluminal tumor may also be confused with food material lodged in the esophagus,but the history will usually readily differentiate them.

Fig. 4. esophagus pattern.

Annular carcinoma with nodularity

of and

the proximal destruction of

thoracic mucosal

186

GOLDSTEIN,

ZORNOZA.

AND

HOPENS

irregular (Fig. 5). With further growth, the neoplasm may encircle the esophagus,creating an annular configuration, or expand further into the lumen and becomeso bulky that it obstructs the esophagus(Fig. 6). The verrucous variant of squamous carcinoma characteristically has a polypoid appearance.24Another variant of the polypoid configuration may closely resemble esophageal varices because of multiple tumor nodules (Fig. 7).25 The infiltrative variety of esophageal cancer

Fig. 5. Double contrast esophagram small polypoid carcinoma originating from of the midesophagus (arrows).

demonstrates the anterior

a wall

affected area is usually irregular and nodular, with mucosal destruction. However, occasionally esophageal cancer may present with atypical and relatively innocuous-appearing radiologic findings. Radiologic

Patterns

The radiologic features of esophagealcancer may be classified into four patterns: annular constricting, polypoid, infiltrative or stenosing, and ulcerative.‘8-2’ Frequently, features of more than one pattern are present. The annular constricting pattern of carcinoma is the most frequent and has a radiologic configuration similar to the annular type of colon cancer (Fig. 4). An overhanging shelf at the proximal and sometimesthe distal margin of the tumor is a typical feature. The narrowed area of lumen is characteristically irregular and ulcerated with absence of recognizable mucosal pattern. The polypoid variety of esophagealcarcinoma is the next most common pattern. When the tumor is small, its origin from the wall of the esophagusmay be seen. Usually the anglesat the attachment are acute and the tumor is somewhat

Fig. 6. Large polypoid carcinoma occupying the distal esophageal lumen in a patient with history of oropharyngeal carcinoma.

most of a previous

ESOPHAGEAL

187

TUMORS

Fi g. 7 . Varicoid carcinoma. sive “Cl ldular carcinomatous esophageal varices.

Esophagram involvement

shows ex simul a lting

may easily be confused with a benign stricture. Since the neoplastic involvement is primarily submucosal, the overlying mucosa is relatively preserved (Fig. 8). The margins of the tumor are often tapered, causing further diagnostic difficulty. Asymmetry of the tumor margins and slight irregularities in the narrowed portion of the esophagus are suggestive signs of carcinoma.

F:ig. 8. Infiltrating squamous a smooth concentric ws causing wit1 R preservation of overlying try 1of the tumor margins.

carcinoma of the cwophanarrowing of the 1 lumen mucosa. There is as iymme-

Endoscopic mucosal biopsy may not yield malignant tissue, since the proximal portion of the tumor may have infiltrated submucosally. The least common radiologic appearance of esophageal cancer is the primary ulcerative type. Ulceration is frequently encountered in all types of esophageal cancer, but in this type virtually all the mass is ulcerated. When seen in profile, the ulcer is meniscoid in shape and surrounded by a similarly shaped tumor ridge (Fig. 9).26 The

GOLDSTEIN,

188

ZORNOZA,

AND

HOPENS

radiologic appearance is similar to the CarmanKirklin malignant gastric ulcer. Detection of a small esophageal cancer, one under 3.5 cm in greatest diameter, is a challenge.” These are usually plaque-like filling defects that may have a small focal ulceration (Fig. 1O).27.28Double contrast esophagography has been shown to be valuable in the detection of these small neoplasms.28*29 Controversy exists as to whether the detection of a small esophageal tumor leads to an improved survival rate following treatment. Conditions Cancer

That Predispose

to Esophageal

There are a number of clinical factors and disease states that are believed to predispose to esophageal cancer.

Fig. 9. Barium study demonstrates a primary ulcerative carcinoma with a large meniscoid ulcer Iarrows) surrounded by a rim of neoplastic tissue in the upper thoracic esophagus.

Fig. 10. Double contrast esophagram shows plaque-like squamous carcinoma along the anterior the esophagus (arrows). The patient was treated squamous carcinoma of the retromolar space earlier.

a small wall of for a 2.5 yr

Fig. expands

11. the

Carcinosarcoma. lumen of the

distal

A large esophagus.

intraluminal

mass

ESOPHAGEAL

189

TUMORS

Fig. 12. Lymphoma. Large nodular folds in the gastric fundus with contiguous involvement of the distal esophagus. (Reprinted with permission from AJR 1977; 128:751-4.1

The vast majority of patients with squamous carcinoma of the head, neck, or esophagus have a history of heavy cigarette or alcohol use.3o The mechanism of cancer production is uncertain in these patients. The frequency of esophageal carcinoma developing in patients with head or neck tumors is at least 8 times greater than would be expected in the general population (Figs. 6 and 10) from chance alone, and the common carcinogenic agent may be related to the cigarettes or alcoho1.3’.32 An increased incidence of squamous carcinoma is also described following chronic corrosive esophageal strictures33 and long-standing achalasia.34 The cancers reported after lye damage occur at the level of the stricture, while those in conjunction with achalasia occur at any level in the esophagus. Squamous carcinoma of the esophagus also occurs as part of a rare hereditary dermatologic condition, tylosis palmaris and plantaris.” The Plummer-Vinson syndrome remains a controversial entity, as does

its possible relationship to cancer of the hypopharynx and cervical esophagus.” In recent years, a number of cases of primary adenocarcinoma have been reported to develop in the columnar-lined esophagus. Occurrence of carcinoma in this setting has been reported to be as high as 8.5% of patients with Barrett esophagus.” Histologic Types The vast majority of esophageal malignancies are squamous cell carcinoma. A variety of other types of malignant tumor occur occasionally, including adenocarcinoma, carcinosarcoma, lymphoma, and metastasis. When an adenocarcinoma is discovered in the distal esophagus, it virtually always has its origin in a proximal gastric carcinoma. As mentioned previously, primary adenocarcinoma of the esophagus usually occurs in the setting of columnar-lined esophagus at the level of a stricture or ulcerati0n.i7.3x

GOLDSTEIN,

Fig. 13. Esophagram esophagus (Reprinted

Histiocytic lymphoma of the esophagus. shows an irregular narrowing of the proximal as well as a large ulceration (arrows). with

permission

from

AJR

1977;

Fig. 14. from a small

Nodular extension adenocarcinoma

ZORNOZA.

AND

HOPENS

into the distal esophagus of the gastric fundus.

128:751-4.)

Carcinosarcoma is an unusual variety of primary malignancy that has both epithelial and connective tissue components.39*40 This tumor characteristically has a bulky polypoid growth pattern and often expands the esophageal lumen (Fig. 11). Small amounts of barium may be trapped on its surface. Other sarcomas of the esophagus are rare.4t Melanoma involving the esophagus is usually on a metastatic basis, but primary melanosarcoma has been reported.42 The esophagus is the alimentary canal organ least commonly involved with lymphoma. Any histiologic variety of lymphoma may affect the esophagus. A wide spectrum of radiologic appearances may be present, as in other portions of the gastrointestinal tract.43 In our experience, the most frequent configuration is contiguous nodular involvement of the distal esophagus and

stomach (Fig. 12). However, lymphomatous infiltration may occur in any segment of the esophagus (Fig. I 3). Metastases to the esophagus probably occur more often than is recognized clinically or radiologically.44 The tumors that most frequently metastasize to the esophagus include gastric, lung, and breast carcinoma as well as melanoma. Many other primary neoplasms with secondary spread to the esophagus have been documented. Secondary neoplastic involvement of the esophagus usually results from direct extension of an adjacent tumor, as with gastric carcinoma (Fig. 14), or from mediastinal metastases from lung or breast carcinoma (Figs. 15 and 16). Adjacent mediastinal neoplasm may infiltrate the esophageal wall and mimic a benign process or may invade transmurally and resemble a primary esophageal cancer. Metastases to the esophagus

ESOPHAGEAL

TUMORS

Fig. 16. Metastatic breast submucosal infiltration in the elongated smooth narrowing stricture.

Fig. 15. Metastatic lung carcinoma with esophageal invasion. Barium study shows destruction of the midesophageal wall. The appearance is indistinguishable from that of an ulcerating primary esophageal cancer.

may be hematogenous ma.45 Differential

in origin, as with melano-

Diagnosis

Current endoscopic biopsy and cytologic methodology usually enable an unequivocal diagnosis of esophageal cancer. However, if radiologic evaluation is suspicious for neoplasm, an inconclusive endoscopic result should not be accepted without question. Both primary and secondary malignant tumors that are largely submucosal in location often yield negative mucosal biopsies. The major radiologic differential diagnostic considerations include esophagitis, achalasia, benign neoplasm, impacted food, varices, and extrinsic mass.

cancer. There is extensive midesophagus, producing an that closely mimics a benign

Benign stricture due to esophagitis associated with peptic disease or previous corrosive ingestion is the most frequent problem. Stricture is particularly difficult to distinguish from infiltrating carcinoma. The narrowed lumen tends to be smoother and the margins more symmetrical with benign stricture. The vertical length of the narrowing is not a helpful criterion. Abrupt tapering of the distal esophagus from achalasia must also be differentiated from esophagogastric cancer. Intermittent partial opening of the distal esophagus especially in the upright position is helpful in making the diagnosis of achalasia and distinguishing it from malignant tumor. Ulceration associated with peptic disease of the esophagus may also be confused with malignant ulceration (Fig. 17). Filling defects in the esophagus from benign tumor, food material, and varices must be distinguished from polypoidal carcinoma. Benign tumors have already been discussed. An intraluminal filling defect secondary to an impacted

192

GOLDSTEIN,

Fig. 17. Barrett (arrows). The patient

ulcer. was

(A) Esophagram thought to have

demonstrates an ulcerating

a large carcinoma.

food bolus is usually no significant problem because of the clear-cut sudden onset while eating. However, such an episode frequently occurs at the site of a preexisting esophageal lesion. While varices will alter in size and shape with each swallow and with respiratory maneuvers, the tumor nodules with varicoid carcinoma are fixed and unchanging. An extrinsic mass may cause narrowing of the esophageal diameter, but its extraesophageal origin usually is apparent. Neoplastic

Staging

Modern radiology has two roles in the evaluation of esophagealtumors, suggesting and establishing the diagnosis and assessingthe extent of esophagealand extraesophageal involvement. The esophagram itself provides important information about the vertical extent of disease, which is critical to treatment planning. In partic-

ulceration (B) After

with nodularity medical therapy

ZORNOZA,

in the midthoracic the ulcer is completely

AND

HOPENS

esophagus healed.

ular, double contrast esophagography often greatly aids accurate assessmentof the tumor limits by most effectively demonstrating the esophagealmucosal surface and by giving information about esophageal wall distensibility.29 Similarly, careful fluoroscopic observation of altered peristalsis and limited distensibility may give information regarding the superior and inferior tumor margins.46Parenteral injection of a spasmolytic agent in association with esophagography occasionally aids evaluation of the inferior extent of a carcinoma by improving filling of the more relaxed esophagusdistal to an obstructing lesion.47 Longitudinal extension from an esophageal cancer often occurs via submucosal lymphatics. Frequently the lymphatic spread extends beyond the gross radiologic and pathologic tumor margins. Such submucosalextension may not be possibleto detect unlesssecondary implantation

ESOPHAGEAL

193

TUMORS

sites are present. Similar to the evaluation of the colon, the entire esophagus should be carefully examined when a tumor is discovered. Synchronously occurring multiple esophageal carcinomas are well documented (Fig. 18).48 Extension of tumor into the mediastinum most frequently has occurred by the time the esophageal carcinoma is diagnosed. Because of the critical anatomic location of the esophagus and

Fig. 19. Perforation of an esophageal cancer, extravasation of contrast into the mediastinum subcarinal region.

Fig. 18. Double large constricting esophagus. A second the distal esophagus

contrast esophagram demonstrates carcinoma of the proximal thoracic small nodular carcinoma is present (arrows).

causing in the

a in

Fig. 20. Frontal ening of the right Fig. 4.

chest radiograph demonstrates thickparatracheal area. Same patient as

194

GOLDSTEIN,

ZORNOZA.

AND

I-IOPENS

Fig. 21. CT of the upper mediastinum of the patient shown in Fig. 9. There is an apparent double esophageal lumen caused by the ulcer (white arrow). A large soft tissue mass is demonstrated in the left paraesophageal and prevertebral areas (black arrows).

its lack of a serosal layer to serve as a barrier, direct spread may occur into the contiguous mediastinum, tracheobronchial tree, aorta, pericardium, mediastinal pleura, and lung. Esophagography may demonstrate local extraesophageal spread by showing free perforation into the mediastinum (Fig. 19) or communication with

Fig. 22. Infradiaphragmatic extension of esophageal carcinoma. Large gastric submucosal metastasis in a patient with treated squamovs carcinoma of the midthoracic esophagus.

the tracheobronchial tree. Chest radiography may show adenopathy or evidence of invasion of the mediastinal structures in well over a third of the cases (Fig. 20).29 Contrast azygography and pneumomediastinography have been utilized in some centers to define mediastinal extension and thereby stage esophageal carcinoma.3’q50 Recent-

ESOPHAGEAL

TUMORS

Fig. 23. CT of the upper abdomen in a patient with esophageal cancer. A large perigastric metastatic mass is present larrowsl.

ly, attention has been directed to CT as an accurate method of staging esophageal carcinoma (Fig. 2 I).” Depending on the site of the primary tumor in the esophagus, lymphatic spread may involve supraclavicular, mediastinal, or subdiaphragmatic nodes and other structures. Esophageal carcinoma arising in the distal half of the thoracic esophagus has major lymphatic drainage to subdiaphragmatic nodes.52 Examination

of the stomach and duodenum at the time of the initial barium study occasionally may show evidence of infradiaphragmatic metastasis from an esophageal carcinoma (Fig. 22). The procedure of choice for evaluation of intraabdominal metastases from esophageal cancer is CT (Figs. 23 and 24). Radiologic evaluation of other distant metastases is indicated as clinically warranted. Besides information regarding mediastinal spread, the

Fig. 24. CT showing left paraaortic metastatic adenopathy secondary to infradiaphragmatic tumor extension from an annular carcinoma of the distal esophagus.

196

GOLDSTEIN,

presence of pulmonary metastases may be diagnosed by conventional chest radiography. Pulmonary parenchymal abnormalities due to aspiration or tracheobronchial fistulization may be confused with lung parenchymal metastases. Hepatic imaging by any of several methods may

be utilized for suspected metastases are noted in clinical course in over esophageal carcinoma.53 most often involved.

ZORNOZA,

AND

HOPENS

liver metastases. Bone the later stages of the 5% of patients with The axial skeleton is

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