Volume 6, Issue 3 , Pages 156-158, September 2011
Pharyngeal tonsil manifestation of Langerhans cells sarcoma: A case report and review of the literature
Article Outline
- Abstract
- 1. Introduction
- 2. Case report
- 3. Discussion
- Conflict of interest
- Acknowledgements
- References
- Copyright
Abstract
Langerhans cell sarcoma (LCS) is an extremely rare malignant disease with multi-organ involvement and a poor prognosis. LCS involvement of tonsil has not been previously reported. However, we present herein a 10-year-old Tibetan boy distressed by LCS arising on the pharyngeal tonsil. Owing to local traditions and customs, he endured a long period of treatment with traditional Tibetan medicine which provided only slight relief at best that delayed an accurate diagnosis and scientific medical treatment. Subsequently, based on CT and MRI scans, combined with pathology features positive immunophenotype for the CD1a and S-100 proteins confirmed the diagnosed of LCS. We performed a surgical resection along with a regimen of E-CHOP chemotherapy was prescribed as new protocols. As a result the patient complete remission symptoms and without relapse has been 20 months.
Keywords: Langerhans cell sarcoma, Pharyngeal tonsil, Immunohistochemical, E-CHOP
1. Introduction
Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features with an aggressive spread. LCS is a very rare disease and theoretically shows multi-organ involvement, including the skin, lymph nodes, lung and bone. Patients have a poor prognosis and a small chance of survival, despite conventional combination chemotherapy, surgery, and radiotherapy [1]. LCS involvement of the tonsil has not been previously reported, and there is a lack of knowledge regarding the prognosis and therapeutic guidelines. This report describes the medical history and histology with immunohistochemical features of the case. An E-CHOP regimen as a new chemotherapeutic protocol was administered post-operatively. A brief review of the relevant literature that might contribute to recognition and management of LCS is provided.
2. Case report
A 10-year-old Tibetan boy was taken to the Traditional Tibetan Medicine Hospital of the Tibetan Autonomous Region (China) for evaluation of a sore throat, dysphagia to solid foods, and an intermittent, unexplained low-grade fever for approximately 5 months. Upon physical examination, a 2.5
cm×3.2cm painful, glossy, nodular mass was noted on the right pharynx spreading to the tonsil. A neck examination revealed no enlarged lymph nodes. He received traditional Tibetan medicine treatment for >4 months. He experienced little relief and the mass did not shrink in size. He was admitted to a local Western-style hospital that performed a computed tomography (CT) scan which revealed a 4cm×4cm×3.5cm middle-density tumor on the right pharynx (Fig. 1). The pathologic examination revealed an inflammatory reaction, which did not respond to antibiotics. Finally, the patient was sent to the West China Hospital for further diagnosis and treatment. A magnetic resonance image (MRI) revealed abnormal signals in the right pharyngeal area and locally invading the parapharyngeal space with a few lymph nodes in the sub-maxillary space (Fig. 1). A surgical resection was performed. The partial neoplastic tissue was sent for frozen section. The pathologic results revealed malignant cytologic features, but a definitive diagnosis was not rendered. Intra-operatively, the right tonsil was infiltrated with tumor and without distinct borders. Extensive surgical resection with leaving a 1cm free margin from the pharyngeal mucosa, as well as a total right tonsillectomy. Histologically, the tumor cells showed diffuse infiltrative growth into the tonsillar fossa and characterized by a proliferation of enlarged, round cells with folded and indented nuclei (Fig. 2B). The final immunohistochemical results revealed that the tumor cells were positive for anti-CD1a and S-100 antibodies (Fig. 2A and C). Consequently, the diagnosis of LCS was made. Under the care of an oncologist, he was began E-CHOP chemotherapy regimen (etoposide, 70mg on d1–3; vincristine, 1.6mg on d1 and 8; cytarabine, 0.85mg on d1; adriamycin, 45mg on d1; and prednisolone, 60mg on d1–14.) After two cycles, he was leukopenic. The laboratory data on presentation were as follows: white blood cell count (WBC), 2.1×109/L (stabs, 5%; segmenteds, 65%; lymphocytes, 19%; monocytes, 5.6%; eosinophils, 3.2%; and basophils, 1.2%). Following a berbamine infusion, to the WBC count returned to normal. The oncologist suggested low-dose radiation therapy for the resected primary lesion, but the boy's parents declined. We continue to follow-up the boy and there has been no recurrent regional or distant metastasis almost 2 years.
Fig. 1.
MRI and CT scan revealed a mass in the fundus of the right side pharyngeal area and part of parapharyngeal space.
Figure 2.
(A) IHC staining positivity anti-CD1a; (B) HE staining tumor cells nuclei are grooved, indented, folded or lobulated; and (C) IHC strong positivity anti-S100.
3. Discussion
Langerhans cell tumors are neoplasms currently classified by the World Health Organization as Langerhans cell histiocytosis (LCH) and LCS. LCH is a proliferating disorder of Langerhans cells, whereas LCS is a neoplastic proliferation of Langerhans cells that have malignant cytologic features [2]. The diagnosis of LCS relies on malignant cytologic features and a combined typical immunophenotype with consistent expression of CD1a and S-100 proteins, as well as Langerin (CD207). It also has the appearance of a typical Birbeck granule [3]. LCS can occur at any age and occurs more commonly in females. LCS may also involve multiple systems or tissues, such as bone, lung, brain, skin/mucous membranes, lymph nodes, liver, and various other soft tissues. It has rapidly growing and aggressive clinical behavior. Prior to the widespread use of immunohistochemistry studies, many of these tumors were diagnosed as non-Hodgkin's lymphoma or other malignant tumors [4]. Currently, the availability of reliable cell markers, this kind of neoplasm has become more readily recognizable and accurately diagnosed [5]. However, according to Ben-Ezra et al. [6] LCSs are often not easily recognized morphologically as a Langerhans cell derivative, but are diagnosed based on CD1a staining. As well as in a recent study, Verdijk et al. [7] reported the lack of Birbeck granules in Langerhans cells to be associated with a mutation in the Langerin gene. The Langerin gene represents a very specific marker for Langerhans cells and derived tumors, even in Birbeck granules-negative cases [8].
In the present case, local conventional practices dictated that the patient rely on traditional Tibetan medicine whether or not his symptoms were relieved, and facilities for cell markers and immunohistochemistry were unavailable in the hospital in Tibet. Thus, there was a failure to promptly treat the boy at first. Nevertheless, in West China hospital although we did not demonstrate the ultrastructural features, but the histomorphologic and immunohistochemical findings supported the diagnosis of LCS. The knowledge of prognosis and therapeutic guidelines were lacking in rarity of this kind of tumor, thus we should provide a new proposal for the therapeutic plan. A PUBMED search of the years from 1960 to 2010 revealed 14 relevant reports of LCS, but none of the reports included LCS involving the pharynx, shows a poor prognosis and a short survival period, despite conventional combination chemotherapy, surgery, and radiotherapy [9]. Thus, still an optimal treatment strategy or neoadjuvant for LCS must be considered to be under investigation. Chemotherapy is commonly undertaken using CHOP (cyclophosphamide, prednisolone, adriamycin, and vincristine) regimen in a standard dose adapted to the common lymphoma treatment protocols [10]. Whereas we provided thoroughly resected the tumor with wide margins and prescribed the E-CHOP regimen chemotherapy as new protocols in the present case. Fortunately, its seems to be an effective therapy. After surgical resection and two subsequent cycles of chemotherapy, the patient showed no apparent recurrent or metastatic signs >20 months later, without any other adjuvant therapy. Of course the tumor size appears to be an important prognostic factor [11]. In our patient, the tumor was localized with no clinical evidence of distant lymphadenopathy or other organ involvement thus the prognosis was favorable. Although our treatment protocol seems to benefit patients with LCS, but it still needs longer follow-up, as well as more patients treated to confirm the therapeutic effects.
In conclusion, we have reported an unusual case of primary pharyngeal LCS, arising on the right pharyngeal tonsil of 10-year-old child. This case shows the necessity of careful attention to clinical symptoms as well as diagnostic pathology procedures in such a rare case. We treated the patient with E-CHOP in a standard dose for new treatment protocols. Although the post-operative follow-up has been >20 months and not recurrent evidence. However, there are no large studies addressing E-CHOP for this neoplasm. Therefore further reports are needed to confirm its therapeutic effect. So a careful long-term follow-up is mandatory.
Conflict of interest
Authors declared no conflict of interest.
Acknowledgements
The patient and his family were informed that data from the case would be submitted anonymously for publication, and they gave their consent.
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PII: S1871-4048(10)00049-3
doi:10.1016/j.pedex.2010.06.004
© 2010 Elsevier Ireland Ltd. All rights reserved.
Volume 6, Issue 3 , Pages 156-158, September 2011
