AIRP Best Cases in Radiologic- Pathologic Correlation

Note: This copy is for your personal non-commercial use only. To order presentation-ready copies for distribution to your colleagues or clients, contact us at www.rsna.org/rsnarights. GASTROINTESTINAL IMAGING 2035 AIRP Best Cases in Radiologic- Pathologic Correlation Spindle Cell Carcinoma of the Esophagus 1 EDITOR S NOTE Everyone who has taken the course in radiologic pathology at the Armed Forces Institute of Pathology (AFIP) remembers bringing beautifully illustrated cases for accession to the Institute. The long-standing and excellent AFIP course in radiologic pathology has transitioned under the auspices of the American College of Radiology to a new home in Silver Spring, Md, entitled the American Institute for Radiologic Pathology (AIRP). In recent years, the staff of the Institute has judged the course s best cases by organ system, and recognition is given to the winners on the last day of the class. With each issue of RadioGraphics, one or more of these cases are published, written by the winning resident. Radiologic-pathologic correlation is emphasized, and the causes of the imaging signs of various diseases are illustrated. Piotr Sadej, MD Rick I. Feld, MD Adam D. Toll, MD Juan P. Palazzo, MD History A 58-year-old man presented with increasing epigastric pain, difficulty swallowing, and weight loss of 8 pounds over several weeks. Approximately 1 year and 3 months earlier, he received simultaneous diagnoses of stage IVb classic type Hodgkin lymphoma, with metastases to the liver, spleen, and bones, and stage II squamous cell carcinoma of the left vocal cord. At the time of presentation, the patient was in remission after undergoing chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine and modified neck dissection to treat the laryngeal carcinoma. No specific observations had been made at a recent physical examination. Imaging Findings Three months earlier, when he was asymptomatic, the patient had undergone follow-up computed tomography (CT) of the chest, abdomen, and pelvis to evaluate for lymphoma. Both oral (Gastrografin; Bracco Diagnostics, Princeton, NJ) and intravenous (Ultravist 300; Bayer HealthCare Pharmaceuticals, Wayne, NJ) contrast material were used, and the findings were negative (Fig 1a). Subsequently, his other symptoms (pain, dysphagia, and weight loss) were evaluated with oral contrast material enhanced CT of the abdomen and pelvis; at that study, only the lower thorax was imaged, and a distended, low-attenuation, presumably fluidfilled distal esophagus was noted (Fig 1b). No other abnormalities were seen. The next day, upper endoscopy was performed to further evaluate epigastric pain, RadioGraphics 2011; 31:2035 2039 Published online 10.1148/rg.317105214 Content Codes: 1 From the Departments of Radiology (P.S., R.I.F.) and Pathology (A.D.T., J.P.P.), Thomas Jefferson University Hospital, 111 S 11th St, Philadelphia, PA 19107. Received October 4, 2010; revision requested October 19 and received November 22; accepted November 24. All authors have no financial relationships to disclose. Address correspondence to P.S. (e-mail: Piotr.Sadej@jeffersonhospital.org). RSNA, 2011 radiographics.rsna.org

2036 November-December 2011 radiographics.rsna.org Figure 1. (a) Axial oral and intravenous contrast-enhanced CT image, obtained in the portal venous phase 3 months earlier, shows the distal esophagus, which appears normal. (b) Axial unenhanced CT image, obtained at the time of presentation, shows the distal esophagus, which is expanded by low-attenuation material (arrow) that was interpreted as fluid. and a friable mass that occupied most of the distal esophagus was seen (Fig 2). Biopsy was performed. Dedicated oral and intravenous (Optiray 320; Mallinckrodt, Hazelwood, Mo) contrastenhanced CT of the chest was performed 1 day after endoscopy and depicted a large lowattenuation (12 HU) mass in the distal esophagus (Fig 3). The area of esophagus proximal to the endoluminal mass was not dilated, the remaining mediastinal structures were uninvolved, and no enlarged mediastinal lymph nodes were seen. Surface-rendered endoluminal reconstructions depicted the polypoid nature of the mass (Fig 4). An upper gastrointestinal series performed to evaluate for obstruction depicted a polypoid mass in the distal esophagus (Fig 5). The proximal esophagus was mildly dilated; however, barium was able to pass through this abnormal segment and into the stomach. Because of the rapid clinical manifestation and fluoroscopy findings, a diagnosis of spindle cell carcinoma was suggested. Figure 2. Endoscopic image shows a large friable mass that occupies almost the entire distal esophagus. Pathologic Evaluation Laparoscopic esophagectomy with cervical esophagogastrostomy was performed, and an ovoid, partially mobile 9.6-cm mass was seen 8 cm proximal to the gastroesophageal junction. The mass was connected to the esophageal mucosa by a 0.5-cm fibrous pedicle (Fig 6a).

RG Volume 31 Number 7 Sadej et al 2037 Figure 3. Axial (a) and sagittal (b) oral and intravenous contrast-enhanced CT images, obtained in the portal venous phase, show that the low-attenuation material in the distal esophagus is actually an ovoid mass (straight arrow in b). The portion of the esophagus proximal to the mass is not dilated (arrowheads in b), and an aberrant right subclavian artery (curved arrow in b) is incidentally seen posterior to the proximal esophagus. Figure 4. Surface-rendered endoluminal reconstructed image obtained from chest CT image data (cf Fig 3) shows a polypoid mass within the region of the distal esophagus. The stalk that attaches the mass to the esophagus is not visualized. Arrow = right mainstem bronchus. Figure 5. Radiograph (left posterior oblique position) obtained at a singlecontrast barium swallow examination shows a polypoid mass expanding the distal esophagus and the classic cupola sign at the proximal interface of the tumor (arrowheads) with the esophageal lumen. Barium is seen distal to the mass, a finding indicative of only partial obstruction.

2038 November-December 2011 radiographics.rsna.org At the time of surgery, frozen sections of the proximal and distal margins, as well as a representative section of tumor, were obtained. The sectioned mass had a yellow-tan, partially gelatinous cut surface (Fig 6b). No gross invasive component was present. Both margins were free of neoplasia. Histologic analysis of the tumor demonstrated a neoplasm that consisted of two components: a minor squamous component and a larger spindle cell component, with focal areas of rhabdomyoblastic differentiation (Fig 7). In situ squamous carcinoma was identified, excluding a metastatic process at this site. Of 11 lymph nodes, one demonstrated metastatic disease. At immunohistochemical analysis, vimentin was expressed in the spindle cell components, and cytokeratin marker AE1-AE3 was expressed in both the squamous and spindle cell components. Sarcomatoid carcinoma is considered an epithelium-derived tumor, with metaplastic mesenchymal differentiation. Thus, epitheliumassociated markers, including cytokeratin marker AE1-AE3, are expected to be expressed in both the carcinomatous and sarcomatous areas. Expression of the mesenchymal-associated marker vimentin is expected only in the sarcomatous areas. The final pathologic diagnosis was invasive sarcomatoid carcinoma, with a note that this lesion also may be called a carcinosarcoma or a spindle cell or metaplastic carcinoma. Discussion Spindle cell carcinoma of the esophagus is rare, accounting for 0.5% 2.8% of all esophageal tumors (1 3). It was first described by Virchow in 1865 as a tumor consisting of both epithelial and spindle cell components and thus was termed carcinosarcoma. Over the years, its name has evolved as more was learned about its origin and behavior; it has also been called pseudosarcoma, polypoid squamous carcinoma, pseudosarcomatous carcinoma, and metaplastic carcinosarcoma, among others (1 4). Initially, it was thought that the sarcomatous component was a reaction to malignant epithelial cells (5). At this time, it is accepted that the tumor originates from an epithelial cell with divergent bidirectional differentiation. Both the epithelial and sarcomatous components of the tumor can metastasize (1,3,6). Figure 6. (a) Photograph of the gross esophageal specimen shows an ovoid solid mass in the distal esophagus that is attached to the esophageal wall by a pedicle (arrow). (b) Photograph of the sectioned mass shows a yellow-tan internal structure. Spindle cell carcinoma usually affects middleaged men (male-to-female ratio, 4:1 to 9:1) (3,7). At least one study reported an association with a strong history of alcohol consumption and cigarette smoking (4). Presenting symptoms are nonspecific; the most common are dysphagia and weight loss, followed by pain (1,7). Most spindle cell tumors (more than 90%) arise from the middle and distal esophagus (1,2,4,7). Because of its intraluminal location and rapid growth (doubling time, 2.2 5 months), patients typically present relatively early in the disease process (2,8). Therefore, rates of invasion of the esophageal wall are lower than those for pure squamous cell esophageal carcinomas (7). Rates of locoregional lymphatic spread and distal hematogenous metastasis, most often to the lung and liver, are reported to be 38% 50% (1,7). At barium swallow examination, spindle cell carcinoma typically manifests as an intraluminal polypoid mass that expands the esophageal lumen, with an average diameter of about 8 cm (1,3,7). The surface of spindle cell carcinoma

RG Volume 31 Number 7 Sadej et al 2039 Figure 7. Photomicrograph (original magnification, 10; hematoxylin-eosin stain) of a specimen of the esophageal tumor shows a biphasic neoplasm with both keratinizing squamous carcinoma (arrow) and spindle cell carcinoma (arrowhead) components. may be smooth, lobulated, or scalloped, and a cupola, or domelike shape, is usually seen at the proximal interface with the esophageal lumen (3). Focal ulcerations are rare. Despite its substantial size, spindle cell carcinoma seldom obstructs the flow of barium into the stomach, as in our case (4). The attachment of the pedicle to the esophageal wall is usually not depicted at fluoroscopy. At CT, spindle cell tumor is seen as an expansile, low-attenuation, intraluminal mass. Focal esophageal wall thickening at the attachment site may be present (4). However, the role of CT is not to evaluate the intraluminal component of the tumor; rather, it is to investigate possible extraesophageal invasion and distant metastases. The differential diagnosis for a polypoid intraluminal esophageal mass includes intraluminal benign masses such as fibrovascular polyp, myofibroma, pedunculated lipoma, and leiomyoma and malignancies such as adenocarcinoma of the esophagus (which may rarely manifest as a solitary polypoid mass), leiomyosarcoma, fibrosarcoma, rhabdomyosarcoma, melanoma, oat cell carcinoma, and lymphoma. Squamous cell carcinoma of the esophagus also may rarely manifest as a polypoid mass. It is difficult to distinguish between these lesions on the basis of radiologic and endoscopic findings alone, and histologic analysis is almost always necessary (1,4). Because spindle cell tumor is so rare, its treatment and prognosis are not well defined. Esophageal resection with gastric pull-through is the most accepted treatment option. The 5-year survival rates of patients with spindle cell carcinoma, at slightly more than 20%, are similar to those of patients with squamous cell carcinoma (1,7). In our case, the patient is alive and has been diseasefree for over 1.5 years. References 1. Iascone C, Barreca M. Carcinosarcoma and pseudosarcoma of the esophagus: two names, one disease comprehensive review of the literature. World J Surg 1999;23(2):153 157. 2. Madan AK, Long AE, Weldon CB, Jaffe BM. Esophageal carcinosarcoma. J Gastrointest Surg 2001;5(4):414 417. 3. Olmsted WW, Lichtenstein JE, Hyams VJ. Polypoid epithelial malignancies of the esophagus. AJR Am J Roentgenol 1983;140(5):921 925. 4. Agha FP, Keren DF. Spindle-cell squamous carcinoma of the esophagus: a tumor with biphasic morphology. AJR Am J Roentgenol 1985;145(3): 541 545. 5. Lane N. Pseudosarcoma (polypoid sarcoma-like masses) associated with squamous-cell carcinoma of the mouth, fauces, and larynx; report of ten cases. Cancer 1957;10(1):19 41. 6. Osamura RY, Shimamura K, Hata J, et al. Polypoid carcinoma of the esophagus: a unifying term for carcinosarcoma and pseudosarcoma. Am J Surg Pathol 1978;2(2):201 208. 7. Iyomasa S, Kato H, Tachimori Y, Watanabe H, Yamaguchi H, Itabashi M. Carcinosarcoma of the esophagus: a twenty-case study. Jpn J Clin Oncol 1990;20(1):99 106. 8. Sasajima K, Taniguchi Y, Morino K, et al. Rapid growth of a pseudosarcoma of the esophagus. J Clin Gastroenterol 1988;10(5):533 536.