Abstract
Intro
Langerhans cell histiocytosis (LCH) is a rare disease involving the proliferation of histiocytes in one or more organ systems. The presentation of LCH is more common in the pediatric population and rarely occurs within the adult population. Isolated lymph node involvement is an extremely rare presentation of the disease. The authors present a case of isolated lymph node LCH in an otherwise healthy adult female and discuss management strategies for these unusual findings.
Methods
A case report of a patient at a tertiary care facility in June 2014 is discussed.
Results/Discussion
The diagnosis, clinical course, and management strategies for isolated adult LCH are discussed and reviewed.
Conclusion
Langerhans cell histiocytosis rarely presents with isolated lymph node involvement in adults. Management and surveillance algorithms are discussed in this unusual case of seemingly benign lymphadenopathy.
1
Introduction
Langerhans cell histiocytosis (LCH) is a rare disease involving the proliferation of histiocytes in one or more organ systems. The presentation of LCH is more common in the pediatric population and rarely occurs within the adult population. Clinical manifestations of the disease are variable, ranging from single organ involvement to disseminated disease. Common sites of involvement among adults include bone, skin, hypothalamus, liver, and lung. Isolated lymph node involvement is rare, with one large study of 314 patients showing only 7% incidence of lymph node involvement at all. Two percent of patients in that study presented with head/neck lymphadenopathy as the dominant presenting feature, and only one patient had lymphatic system involvement alone . The authors present a case of isolated lymph node LCH in an otherwise healthy adult female and discuss management strategies for these unusual findings.
2
Case history
A 20-year-old female was referred to the ENT department with a two-year history of postauricular lymphadenopathy and intermittent tenderness to palpation. The patient denied any fevers, chills, or weight loss and had an otherwise unremarkable medical history. Physical exam revealed a 5 mm palpable mass in the right postauricular region without tenderness or skin involvement. Ultrasound imaging revealed two enlarged postauricular lymph nodes with normal morphology and a measurement axis of 1.3 cm. The patient was subsequently treated with a course of antibiotics without any decrement in size, and the patient elected to undergo excision of the palpable lymph node due to continued tenderness as well as for diagnostic purposes. She was taken to the operating room and underwent a successful and uneventful excisional biopsy but pathology review showed the lymph node to be consistent with LCH.
2
Case history
A 20-year-old female was referred to the ENT department with a two-year history of postauricular lymphadenopathy and intermittent tenderness to palpation. The patient denied any fevers, chills, or weight loss and had an otherwise unremarkable medical history. Physical exam revealed a 5 mm palpable mass in the right postauricular region without tenderness or skin involvement. Ultrasound imaging revealed two enlarged postauricular lymph nodes with normal morphology and a measurement axis of 1.3 cm. The patient was subsequently treated with a course of antibiotics without any decrement in size, and the patient elected to undergo excision of the palpable lymph node due to continued tenderness as well as for diagnostic purposes. She was taken to the operating room and underwent a successful and uneventful excisional biopsy but pathology review showed the lymph node to be consistent with LCH.
3
Discussion
LCH is a rare disease of unknown etiology, characterized as a proliferative disorder of Langerhans cells (LCs) in one or more organ systems. LCs are bone marrow-derived dendritic cells, which normally reside in the skin and lymph nodes . Definitive diagnosis of LCH as set forth by the Histiocyte Society classification criteria requires the presence of CD1a or CD207 antigenic determinants on the surface of cells in question . LCH predominantly presents in the pediatric population and little information is available concerning LCH and adults; only a few reports have been published involving a series of adult patients diagnosed with LCH . In a report published in 2003 based on data from the International Registry of the Histiocyte Society, the mean age at onset of disease was 33 years. This registry data included only adults 18 years or older, totaling 274 patients from 13 different countries, collected between January 2000 and June 2001 .
LCH may present with a variety of symptoms based on the organ systems involved. The most common sites of presentation amongst reports on adult LCH include bone, skin, and lung manifestations . Symptoms may include bone pain, rash (often on the scalp), dyspnea, chronic cough, or spontaneous pneumothorax. Overall, lymph node involvement is infrequent, and most often manifests as enlargement of one or more the cervical nodes. In the IRHS report, the histological diagnosis of LCH was made from lymph nodes in only 6.3% of patients . Additionally, Howarth et al. reported only 1 of 104 patients with non-osseous single-system LCH disease presenting with lymph node infiltration, and only 21 of 314 patients had any lymph node involvement at all . Histopathologically, tissues involved in LCH often show effacement of the nodal architecture and sinuses markedly distended by histiocytes. Immunohistochemical staining of the histiocytes are positive for CD1a, S-100, and CD-68 ( Figs. 1–4 ).
