Ann Thorac Surg 2008;85:1102-1104. doi:10.1016/j.athoracsur.2007.09.003
© 2008 The Society of Thoracic Surgeons
Case Reports
Esophageal Carcinosarcoma With Basaloid Squamous Cell Carcinoma and Osteosarcoma
Jung-Jyh Hung, MDa,b,
Anna Fen-Yau Li, MD, PhDc,
Jung-Sen Liu, MD, PhDb,
Yu-Sen Lin, MDd,
Wen-Hu Hsu, MDd,*
a Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan
b Department of Surgery, Cathay General Hospital, School of Medicine, Fu Jen Catholic University, Taipei, Taiwan
c Department of Pathology, Taipei Veterans General Hospital, and School of Medicine, National Yang-Ming University, Taipei, Taiwan
d Division of Thoracic Surgery, Department of Surgery, Taipei Veterans General Hospital, and School of Medicine, National Yang-Ming University, Taipei, Taiwan
Accepted for publication September 4, 2007.
* Address correspondence to Dr Hsu, Division of Thoracic Surgery, Department of Surgery, Taipei Veterans General Hospital, No. 201, Sec. 2, Shih-Pai Rd, Taipei, 112, Taiwan (Email: whhsu{at}vghtpe.gov.tw).
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Abstract
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Carcinosarcoma of the esophagus is a rare malignant neoplasm. It often presents as a large intraluminal polypoid tumor with early onset of symptoms resulting in prompt diagnosis. We present here the first case of a carcinosarcoma of the esophagus that had a basaloid squamous cell carcinoma component in addition to the osteosarcoma and without a transitional zone. A 57-year-old man was diagnosed with a polypoid tumor in middle third of the esophagus causing dysphagia. Local recurrence and solitary pulmonary metastasis occurred 16 and 30 months after initial excision of the tumor, respectively. Prompt and aggressive surgical resection is mandatory for carcinosarcoma of the esophagus.
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Introduction
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Carcinosarcoma of the esophagus is a rare malignant neoplasm consisting of both carcinomatous and sarcomatous elements. Its reported incidence is approximately 2% of all esophageal neoplasms [1]. Common symptoms include dysphagia, odynophagia, and body weight loss, similar to those caused by squamous cell carcinoma. However, because of typical presentation as a polypoid mass, the symptoms manifest earlier. We present here the first case of esophageal carcinosarcoma that had basaloid squamous cell carcinoma and osteosarcoma components.
A 57-year-old man was admitted to the thoracic surgery department because of dysphagia for 2 months. Medical history included hypertension, diabetes mellitus, and an episode of cerebrovascular accident 6 months before admission. Physical examination revealed no abnormal physical findings except for weakness of the right extremities. Barium contrast study and esophagogastroduodenoscopy demonstrated a polypoid mass 9 cm in length with a narrow stalk in the middle third of the esophagus (Fig 1). Multiple endoscopic biopsies yielded no pathologic diagnosis but fibrous exudates. Computed tomography (CT) of the chest revealed a large esophageal mass without adjacent organ invasion or lymphadenopathy. Whole-body bone scan and abdominal sonogram showed no evidence of distant metastasis.
The patient underwent myotomy of the esophagus and tumor excision. Intraoperatively, a polypoid tumor, 9 x 2.5 x 3.5 cm, with a pedicle arising from the right posterolateral wall of the middle third of the esophagus, was found (Fig 2). Frozen section revealed squamous cell carcinoma. Subtotal esophagectomy was suggested after the frozen section, but the families refused because of the high operative risk from the recent cerebrovascular accident. Mediastinal radical lymph node dissection was performed instead.
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Fig 2. Macroscopic appearance of the tumor. (A) Macroscopic findings of the resected specimen showing a 9.5 x 4 x 2.7 cm lobulated mass with a short stalk. (B) Longitudinal section of the tumor.
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Permanent pathology examination revealed basaloid squamous cell carcinoma components in addition to the osteosarcoma components but without a transitional zone (Fig 3). A gross examination of the resected specimen showed a large polypoid and lobulated tumor measuring 9.5 x 4 x 2.7 cm with a short stalk (Fig 2A). The cross-section revealed soft, grayish, and focal hemorrhage (Fig 2B). Microscopically, the tumor consisted of epithelial and mesenchymal components. The epithelial components included squamous cell carcinoma in situ, as well as a basaloid squamous cell carcinoma (Fig 3A). The sarcomatous component revealed osteosarcoma (Fig 3B). On immunohistochemistry analysis, cells of epithelial origin were positive for cytokeratin (Fig 3C). The cells identified with cytokeratin stain also showed positive staining for Bcl-2 (Fig 3D). The excised lymph nodes did not harbor tumor cell. Pathology staging was pT2N0M0, stage IIA. The patient was discharged in stable condition and followed-up by chest CT at the outpatient department at an interval of 3 months after surgery.
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Fig 3. Histopathologic examination. (A) Squamous cell carcinoma in situ and invasive basaloid squamous cell carcinoma (hematoxylin and eosin stain, x40). (B) Osteosarcoma component (hematoxylin and eosin stain, x100). (C) Cells of epithelial origin identified with cytokeratin stain (keratin stain, x100). (D) Cells identified with cytokeratin stain in (C) also showed positive staining for Bcl-2 (Bcl-2 stain, x200).
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Dysphagia developed 16 months after surgery. Esophagogastroduodenoscopy demonstrated an ulcerative tumor located at the previous operative site. Biopsy revealed squamous cell carcinoma. The patient underwent esophagectomy with a gastric pull-up reconstruction and cervical anastomosis through laparotomy, right thoracotomy, and left cervical incisions. Permanent pathology examination revealed basaloid squamous cell carcinoma. Unfortunately, chest radiogram showed a nodule at the right lower lung field 14 months after esophagectomy. Whole-body positron emission tomography and CT scan revealed a nodule (2.6 x 2.5 x 2.2 cm) located at the right lower lobe with standard uptake value of 7.6. Wedge resection of the nodule was done through right thoracotomy. Pathology revealed metastatic basaloid squamous cell carcinoma. The patient underwent cisplatin, 5-fluorouracil, and leucovorin chemotherapy in the following months.
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Comment
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Carcinosarcoma of the esophagus is usually composed of invasive or in situ squamous cell carcinomas, or both, with a sarcomatous component [2]. Esophageal carcinosarcoma consisting of basaloid squamous carcinoma was first reported by Ohtaka and colleagues [3] in 2002. Amatya and coworkers [4] reported a case of esophageal carcinosarcoma consisting of basaloid squamous carcinoma and rhabdomyosarcoma in 2004. We present here a case of carcinosarcoma of the esophagus that had basaloid squamous cell carcinoma elements in addition to the osteosarcoma without a transitional zone.
Basaloid squamous cell carcinoma, originally described by Wain and colleagues [5] in 1986, is an uncommon variant of squamous cell carcinoma that usually occurs in the upper aerodigestive tract. Basaloid squamous cell carcinoma of the esophagus is extremely rare, with a reported incidence of less than 2% [6]. This is characterized by higher proliferative activity and higher apoptotic indices as compared with typical esophageal squamous cell carcinoma [7]. However, the survival rate of patients with either type of carcinoma does not differ significantly [7].
Carcinosarcoma may spread through direct invasion, hematogenous route, or lymphatic circulation. Lymph node metastases occur in approximately 50% of patients. The 5-year survival rate for patients undergoing esophagectomy for carcinosarcoma has been reported as 54% [8]. Despite its polypoid appearance, carcinosarcomas have significant metastatic potential. Moreover, the poor degree of differentiation and the high proliferative activity in basaloid squamous cell carcinoma may lead to the frequent development of distant metastases. The basaloid squamous cell carcinoma component is responsible for the recurrence in the current case.
Esophageal carcinosarcoma should be treated by esophagectomy with adequate lymph node dissection, especially if the tumor consists of a basaloid squamous cell carcinoma component. Polypectomy should be avoided even with a narrow pedicle because of the high incidence of recurrence.
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Acknowledgments
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The authors are grateful to Drs William D. Travis, David S. Klimstra, and Jinru Shia of the Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York, for their confirmation of the pathologic diagnosis. They also thank Dr Wen-Juei Jeng for her contribution to this article.
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References
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- Kuhajda FP, Sun TT, Mendelsohn G. Polypoid squamous carcinoma of the esophagus Am J Surg Pathol 1983;7:495-499.[Medline]
- Ohtaka M, Kumasaka T, Nobukawa B, et al. Carcinosarcoma of the esophagus characterized by myoepithelial and ductal differentiations Pathol Int 2002;52:657-663.[Medline]
- Amatya VJ, Takeshima Y, Kaneko M, Inai K. Esophageal carcinosarcoma with basaloid squamous carcinoma and rhabdomyosarcoma components with TP53 mutation Pathol Int 2004;54:803-809.[Medline]
- Wain SL, Kier R, Vollmer RT, Bossen EH. Basaloid squamous carcinoma of the tongue, hypopharynx and larynx Hum Pathol 1986;17:1158-1166.[Medline]
- Abe K, Sasano H, Itakura Y, Nishira T, Mori S, Nagura H. Basaloid-squamous carcinoma of the esophagus: a clinicopathologic, DNA ploidy, and immunohistochemical study of seven cases Am J Surg Pathol 1996;20:453-461.[Medline]
- Sarbia M, Verreet P, Bittinger F, et al. Basaloid squamous cell carcinoma of the esophagus. Diagnosis and prognosis. Cancer 1997;79:1871-1878.[Medline]
- Iascone C, Barreca M. Carcinosarcoma and pseudosarcoma of the esophagus: two names, one disease—comprehensive review of the literature World J Surg 1999;23:153-157.[Medline]
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Abstract
Introduction
