In a striking case published in the World Journal of Surgical Oncology, a 63-year-old male with a history of smoking presented with hemoptysis and was diagnosed with a rare bipartite combined esophageal tumor, consisting of both sarcomatoid carcinoma and small cell carcinoma. Despite an initially successful surgery, early recurrence with distant metastases led to a swift course adjustment to palliative chemotherapy. This case underscores the complexity of managing combined esophageal tumors and the importance of considering their aggressive behavior in therapeutic decision-making.
DOI: 10.1186/s12957-019-1623-7
Introduction
Bipartite combined esophageal tumors are rare entities characterized by the coexistence of two histologically distinct malignancies within the same tumor. With the majority of esophageal tumors presenting as a single histological type, the management and prognosis of these unique combined tumors are not extensively documented in the literature.
Case Presentation
In a noteworthy case reported in the World Journal of Surgical Oncology, a 63-year-old Chinese male, with a smoking history, came to medical attention after experiencing hemoptysis against a backdrop of dysphagia and odynophagia persisting for one month. Diagnostic endoscopy revealed an exophytic tumor in the esophagus that was prone to contact bleeding, and the biopsy identified it as a high-grade malignant growth. However, immunohistochemistry failed to clarify the tumor’s lineage, with differentials spanning sarcomatoid carcinoma to malignant undifferentiated sarcoma.
The patient underwent a minimally invasive McKeown’s esophagectomy based on the provisional diagnosis of high-grade esophageal sarcoma. Histology from the surgical specimen displayed a bipartite combined tumor comprised of 70% poorly differentiated spindle cell squamous carcinoma and 30% small cell carcinoma. This diagnosis prompted the initiation of adjuvant chemotherapy post-operatively to address the small cell carcinoma component.
Unfortunately, a mere four months following the operation, a computed tomography scan unveiled metastases spread to the lung, pleura, liver, and thoracic nodes. A lung nodule biopsy reaffirmed the presence of small cell neuroendocrine carcinoma akin to the esophagus’s small cell component.
The patient then commenced palliative chemotherapy with carboplatin and etoposide, though etoposide was abandoned after the emergence of asymptomatic bradycardia. The protocol was adjusted to combine carboplatin with irinotecan for a series of five cycles, which, upon completion, resulted in the complete response of lung and liver metastases, as well as no observable local or distant recurrence.
Discussion
The aggressive nature and early recurrence post-surgery in this case parallel the behavior of primary esophageal small cell carcinoma, noted for its poor prognosis. The challenges of preoperative confirmation of combined tumors lie in the biopsy’s frequent failure to yield both components, which can potentially impact the treatment strategy. As presented, the more aggressive small cell component took precedence in directing therapy, aligning with the notion that platinum-based chemotherapy, not surgery, should be the cornerstone for managing small cell carcinoma.
Management Considerations
With surgery being the preferred treatment modality for localized esophageal squamous cell carcinoma, the presence of a small cell component complicates decision-making. The rapid postoperative progression in this case emphasizes the need to tailor treatment plans based on the predominant tumor biology. Palliative chemotherapy aims to manage symptoms and prolong survival, with patient response dictating subsequent steps.
Conclusion
This case illustrates the necessity for a better understanding of bipartite combined esophageal tumors, especially given the difficulties in distinguishing the tumor types preoperatively and determining the optimal therapeutic approach. Continued research and documentation of such cases are essential in creating evidence-based guidelines to improve patient outcomes for these rare tumors.
References
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Keywords
1. Bipartite combined esophageal tumor
2. Esophageal sarcomatoid carcinoma
3. Small cell carcinoma esophagus
4. Postoperative esophageal cancer recurrence
5. Chemotherapy esophageal sarcoma