Case report
Department of Otorhinolaryngology-Head and Neck Surgery of West China Hospital of Sichuan University, 37# Guoxuexiang, Chengdou, Sichuan, China
Department of Otorhinolaryngology of Peoples Hospital of The Tibet Autonomous Region, 18# Linkor Northern Road, Lhasa, China
A R T I C L E I N F O
A B S T R A C T
Article history:
Received 1 December 2009
Received in revised form 20 June 2010
Accepted 23 June 2010
Available online 21 July 2010
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 scientic medical treatment.
Subsequently, based on CT and MRI scans, combined with pathology features positive immunophenotype for the CD1a and S-100 proteins conrmed 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.
2010 Elsevier Ireland Ltd. All rights reserved.
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 3.2 cm painful,
glossy, nodular mass was noted on the right pharynx spreading to
157
Fig. 1. MRI and CT scan revealed a mass in the fundus of the right side pharyngeal area and part of parapharyngeal space.
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 boys parents
declined. We continue to follow-up the boy and there has been no
recurrent regional or distant metastasis almost 2 years.
3. Discussion
Langerhans cell tumors are neoplasms currently classied 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-Hodgkins
lymphoma or other malignant tumors [4]. Currently, the
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.
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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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