Extraskeletal soft tissue masses of Langerhans' cell histiocytosis

1996 ◽  
Vol 25 (4) ◽  
pp. 409-412 ◽  
Author(s):  
Mark E. Henck ◽  
E. L. Simpson ◽  
Richard H. Ochs ◽  
Joseph L. Eremus
2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Anil Gungadeen ◽  
Peter Kullar ◽  
Philip Yates

Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature.Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis. His otological symptoms were completely resolved with the systemic therapy.Conclusions. Otic Langerhans' cell histiocytosis can present in adults. Persistent ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include Langerhans' cell histiocytosis in their differential diagnosis. Management should be with systemic therapy rather than local surgical treatment.


2015 ◽  
Vol 33 (9) ◽  
pp. 603-606
Author(s):  
Cem Onal ◽  
Ezgi Oymak ◽  
Mehmet Reyhan ◽  
Tuba Canpolat ◽  
Ozgur Ozyilkan

2019 ◽  
Vol 24 (6) ◽  
pp. 728-732
Author(s):  
M. Omar Iqbal ◽  
Ashirwad Merve ◽  
Nathalie Galea ◽  
Kristian Aquilina

Tumors of the CNS represent the largest group of solid tumors found in the pediatric patient population. Langerhans cell histiocytosis (LCH) is an inflammatory lesion that may present in bone and/or soft tissue, including the CNS. Management depends on the extent of multisystem involvement, which determines resection with or without systemic chemotherapy. The authors report on the case of a child who underwent an open craniotomy for biopsy of a pituitary stalk lesion followed by neuropathological assessment, procedures used to diagnose LCH. The patient then underwent 12 months of systemic chemotherapy with subsequent resolution of the pituitary stalk lesion. Two years following pathological diagnosis, the patient presented with frontal orbital pain at the site of the prior craniotomy. Advanced imaging revealed MRI enhancement and radiotracer uptake of a soft-tissue growth at the frontal burr-hole site and MRI enhancement at a posterior burr-hole site without soft-tissue growth. The patient then underwent open biopsy and curettage that revealed LCH recurrence at the site of prior craniotomy. This case demonstrates that LCH may represent an abnormal reactive clonal proliferation of dendritic cells, rather than a de novo malignant neoplasm that can occur at sites of prior craniotomy despite systemic chemotherapy. The authors advocate close follow-up with contrast-enhanced imaging. Special attention should be given to sites of prior surgical manipulation to avoid missing distant sites of recurrence.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Mimi Zhao ◽  
Limin Tang ◽  
Shiqing Sun ◽  
Jiufa Cui ◽  
Haisong Chen

Abstract Background This study aimed to identify the characteristic radiological signs for the diagnosis of Langerhans cell histiocytosis (LCH) of the bone. Methods We retrospectively studied 82 cases of LCH with bone lesions confirmed by pathology. Clinical and radiological features of the patients were analyzed. Results A total of 64 and 18 patients had single and multiple bone lesions, respectively. With regard to LCH with single bone lesions, 37.5% (24/64) of lesions were located in the skull and presented as bone destruction with or without soft tissue mass. The correct diagnosis rate of these lesions was 60.0% (9/15) in children and adolescents, but was only 22.2% (2/9) in adults. A total of 26.5% (17/64) of the solitary lesions were found in the spine. Of these, 88.2% (15/17) were located in the vertebral body and appeared to have different degrees of collapse, and 66.7% (10/15) of these lesions were correctly diagnosed. Of the unifocal lesions, 21.8% (14/64) were located in other flat and irregular bones and manifested as osteolysis. Only 21.4% (3/14) of these cases were correctly diagnosed. In total, 14.1% (9/64) of the isolated bone LCH lesions were located in the long bones. Of these, 77.8% (7/9) were located in the diaphysis and presented as central bone destruction with or without fusiform periosteal reaction and extensive peripheral edema, of which 42.9% (3/7) were correctly diagnosed before surgery or biopsy. With regard to LCH with multiple bony destructive lesions, 71.4% (10/14) of cases in children and adolescents were correctly diagnosed; however, all four cases among adults were misdiagnosed. Conclusion In all age groups, isolated diaphyseal destruction of the long bone with fusiform periosteal reaction and extensive peripheral edema, vertebra plana of the spine, and bevelled edge of skull defects accompanied by soft tissue masses strongly suggest LCH diagnosis. Moreover, the multiple bone osteolytic destruction in children and adolescents strongly suggests LCH diagnosis. Familiarity with these typical radiological signs of LCH is necessary to decrease misdiagnoses.


1988 ◽  
Vol 5 (4) ◽  
pp. 319-324 ◽  
Author(s):  
Y. Frishberg ◽  
B. Stark ◽  
C. Mor ◽  
M. Mukamel ◽  
I. Yaniv ◽  
...  

2008 ◽  
Vol 33 (3) ◽  
pp. 731-736 ◽  
Author(s):  
Xin-Sheng Peng ◽  
Tao Pan ◽  
Li-Yan Chen ◽  
Gang Huang ◽  
Jin Wang

2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
Sai Gao ◽  
Ruizhi Zhou ◽  
Qi Xu ◽  
Haisong Chen

Objective. To explore the incidence and significance of intra- and extra-osseous edema associated with benign tumors and tumor-like diseases. Methods. Magnetic resonance imaging (MRI) data from 300 benign osseous tumors and tumor-like diseases diagnosed by pathology were retrospectively reviewed. Borderline tumors, cases associated with pathological fractures, and skull lesions were excluded from the study. Bone marrow and soft tissue edema were defined on T2WI with fat suppression on MRI in all cases. The incidence rate of edema in benign tumors and tumor-like diseases was determined using the χ2 test. The preoperative diagnoses were reviewed, and the effect of edema on the differential diagnosis of benign and malignant tumors was analyzed. Results. The incidence rate of bone marrow and soft tissue edema associated with benign tumors and tumor-like diseases was 35.7% (107/300), including 84.4% (27/32) Langerhans cell histiocytosis, 86.4% (19/22) osteoblastoma, 93.9% (31/33) osteoid osteoma, and 85.2% (23/27) chondroblastoma cases. There was no statistically significant difference in the incidence of edema among the four diseases (χ2=1.7, P>0.05). Of 107 cases associated with edema, 49 (45.8%) were misdiagnosed as malignant tumors by MRI preoperatively. Conclusion. Bone marrow and soft tissue edema are a common finding associated with benign bone tumors and tumor-like diseases, and they are frequently detected in Langerhans cell histiocytosis, osteoblastoma, osteoid osteoma, and chondroblastoma.


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