Korean J Radiol.  2013 Jun;14(3):520-524. 10.3348/kjr.2013.14.3.520.

Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence

Affiliations
  • 1Department of Radiology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 137-701, Korea. saim@catholic.ac.kr
  • 2Department of Pediatrics, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 137-701, Korea.
  • 3Department of Hospital Pathology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul 137-701, Korea.

Abstract

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.

Keyword

Langerhans cell sarcoma; Langerhans cell histiocytosis; CT; MR

MeSH Terms

Female
Hepatomegaly/diagnosis
Humans
Infant
Langerhans Cell Sarcoma/*diagnosis
Magnetic Resonance Imaging
Mediastinal Neoplasms/*diagnosis
Neoplasm Recurrence, Local
Skull Neoplasms/*diagnosis
Splenomegaly/diagnosis
Tomography, X-Ray Computed

Figure

  • Fig. 1 Case 1. 11-month-old female infant presented with intermittent fever. A. Contrast-enhanced CT of abdomen shows splenomegaly (about 14 cm) with multifocal low density lesions in spleen (arrows). Soft tissue density lesions are seen in porta hepatis (arrowheads). B. Contrast-enhanced CT of chest shows nodular enlargement of thymus with bilateral pleural effusions. C. Typical histology of Langerhans cell sarcoma with malignant cytological features, such as atypia, hyperchromatic nuclei (H&E, × 400), and prominent nucleoli. D, E. Cells are immunopositive for CD1a (× 400) and S-100 (× 400). F. At 16 months after chemotherapy, follow-up MRI of orbit (fat-suppressed T2-weighted image) shows well-enhancing soft tissue mass extending from anterior skull base (including sphenoid wing and infratemporal fossa) to lateral aspect of right orbit and frontal area (arrow). G. Follow-up CT of orbit shows associated adjacent bony destruction with irregular sharp margin (arrow). Osteolytic lesions involving anterior skull base to lateral aspect of left orbit are also noted (arrowheads).

  • Fig. 2 Case 2. 17-month-old girl presented with intermittent fever. A. Contrast-enhanced CT of abdomen shows huge soft tissue density lesion with heterogeneous enhancement in anterior mediastinum. B. In upper abdomen, soft tissue density lesions encase main portal vein, hepatic artery and splenic artery, without evidence of luminal narrowing (arrows). C, D. At 17 months after chemotherapy, follow-up CT of skull shows multifocal osteolytic lesions including greater wing of left sphenoid bone, and right temporal bone (arrows). E, F. One year after salvage chemotherapy, follow-up contrast-enhanced CT of abdomen shows nodular calcifications along biliary trees.


Cited by  1 articles

A Case of Langerhans Cell Sarcoma Presenting as Submandibular Gland Mass
Geonho Lee, Kunho Song, Ki Wan Park, Bon Seok Koo
Korean J Otorhinolaryngol-Head Neck Surg. 2019;62(9):520-523.    doi: 10.3342/kjorl-hns.2018.00661.


Reference

1. Zhao G, Luo M, Wu ZY, Liu Q, Zhang B, Gao RL, et al. Langerhans cell sarcoma involving gallbladder and peritoneal lymph nodes: a case report. Int J Surg Pathol. 2009. 17:347–353.
2. Lee JS, Ko GH, Kim HC, Jang IS, Jeon KN, Lee JH. Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report. J Korean Med Sci. 2006. 21:577–580.
3. Nakayama M, Takahashi K, Hori M, Okumura T, Saito M, Yamakawa M, et al. Langerhans cell sarcoma of the cervical lymph node: a case report and literature review. Auris Nasus Larynx. 2010. 37:750–753.
4. Luo B, Pian B, Peng Z, Jintao D, Shixi L. Pharyngeal tonsil manifestation of Langerhans cells sarcoma: a case report and review of the literature. Int J Pediatr Otorhinolaryngol Extra. 2011. 6:156–158.
5. Uchida K, Kobayashi S, Inukai T, Noriki S, Imamura Y, Nakajima H, et al. Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen. J Orthop Sci. 2008. 13:89–93.
6. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007. 48:124–131.
7. Donadieu J, Chalard F, Jeziorski E. Medical management of langerhans cell histiocytosis from diagnosis to treatment. Expert Opin Pharmacother. 2012. 13:1309–1322.
8. Caruso S, Miraglia R, Maruzzelli L, Luca A, Gridelli B. Biliary wall calcification in Langerhans cell histiocytosis: report of two cases. Pediatr Radiol. 2008. 38:791–794.
9. Pyun HW, Kim ME, Kim JH. CT and MR findings of langerhans cell histiocytois involving the spleen: a case report. J Korean Radiol Soc. 2002. 46:171–174.
10. Heller GD, Haller JO, Berdon WE, Sane S, Kleinman PK. Punctate thymic calcification in infants with untreated Langerhans' cell histiocytosis: report of four new cases. Pediatr Radiol. 1999. 29:813–815.
11. Yi G, Yoon HK, Kim BK, Kim KA, Choo IW. CT findings of orbital langerhans cell histiocytosis. J Korean Radiol Soc. 2000. 42:841–846.
Full Text Links
  • KJR
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr