Soft tissue Langerhans cell histiocytosis with secondary bone involvement in extremities: evolution of lesions in two patients

Langerhans cell histiocytosis (LCH) is an uncommon disorder of unknown etiology with a wide spectrum of biological behavior. The most common sites of involvement are bone, skin, and lung. While osseous LCH can result in secondary soft tissue involvement, primary soft tissue LCH in extremities with secondary bone involvement is rare, and little is known about its natural course. We report two adult patients with multi-organ system LCH who had primary soft tissue lesions with secondary involvement of adjoining bones in lower extremities, and illustrate the important role played by various imaging modalities in detection and monitoring of treatment response. We also document spontaneous clinical resolution of LCH lesions without therapy, and positive response of these lesions to chemotherapy.     

Bone scintigraphy and somatostatin receptor scintigraphy in pediatric patients with bone involvement in Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a granulomatous disease which can involve multiples sites of the body. Diagnostic imaging is of utmost importance in the management of these patients. Up to now radiographic skeletal survey and bone scintigraphy (BS) have been used to assess bone involvement (both with low specificity). Magnetic resonance imaging (MRI) and CT have been used to assess visceral involvement but with the limitation that they cannot give information about the functional status. Recently somatostatin receptor scintigraphy (SSRS) has been proposed to detect active lesions and to monitor response to treatment. The aim of this study is to assess bone and somatostatin receptor scintigraphy in the detection of bone involvement in LCH in children. Twenty scintigraphies (12 SSRS and 8 BS) were performed in seven patients (3 girls and 4 boys) aged at diagnosis: 18 month-12 years (mean age 6 years). The findings obtained in the scintigraphies were compared with clinical evolution and other imaging techniques. Bone scintigraphy detected all the LCH bone lesions, and discovered one unknown lesion. SSRS scintigraphy visualised the active lesions in 3 patients (clinical and other imaging techniques were also positive). SSRS was negative in one patient classified as disease free and another in clinical remission. SSRS detected 2 new unknown bone lesions, but could not detect LCH bone lesions confirmed in other imaging techniques in 2 patients. Somatostatin receptor and Bone scintigraphy can be used to detect active LCH bone lesions in children and can help to monitor response to treatment. Further studies with more patients are needed to confirm the diagnostic usefulness of these techniques.     

Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases

Objective. Unusual manifestations are occasionally encountered in Langerhans cell histiocytosis and may be a source of confusion. The objective of this study was to determine the frequency of occurrence of the unusual manifestations in our case material. Design and patients. Thirty-four children, average age 4.4 years (range 3 months to 16 years) with 262 skeletal lesions of biopsy-proven Langerhans cell histiocytosis (LCH) were retrospectively reviewed to determine the frequency of occurrence of unusual manifestations defined either as an atypical location of a skeletal lesion or an atypical radiographic appearance of the lesion. Results. Twenty-four unusual lesions were found in this retrospective review. Among these were epiphyseal lesions, transphyseal lesions, extracranial ’button’ sequestra, posterior vertebral arch lesions, dural extension of vertebral lesions, and fluid-fluid levels. The finding of fluid-fluid levels has not previously been described in the radiologic literature. Involvement of unusual sites included clavicles and small bones of the hands and feet. Conclusions. Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis.     

儿童郎格尔汉斯细胞增生症的CT表现（附13例分析 ）

目的 探讨CT对郎格尔汉斯细胞组织细胞增生症（Langerhans cell histocytosis,LCH)造成多脏器损害诊断的价值及限度。方法 分析13例经临床、实验室、病理检查确诊的LCH的CT表现。局限性LCH4例,广泛性LCH9例。全部病例均行颅脑、胸部、肝、脾CT平扫检查,4例行增强检查。结果 男性多于女性,颅穹隆骨破坏依次为颞骨8例、顶骨3例、枕骨2例、额骨1例。CT发现早期骨破坏敏感性高。肿块变化可反映病变由活跃增殖到静止消退的病理过程。下丘脑－垂体轴侵犯影像表现晚于临床。CT可显著LCH不同阶段的肺损害。结论 LCH各脏器损害的影像学表现缺乏特异性。好发部位的典型CT表现可提示诊断,同时对辅助LCH分型、确定治疗方案、估计预后有一定价值。     

儿童郎格尔汉斯细胞组织细胞增生症的CT表现(附13例分析)

目的　探讨CT对郎格尔汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)造成多脏器损害诊断的价值及限度。方法　分析13例经临床、实验室、病理检查确诊的LCH的CT表现。局限性LCH 4例,广泛性LCH 9例。全部病例均行颅脑、胸部、肝、脾CT平扫检查,4例行增强检查。结果　男性多于女性,颅穹隆骨破坏依次为颞骨8例、顶骨3例、枕骨2例、额骨1例。CT发现早期骨破坏敏感性高。肿块变化可反映病变由活跃增殖到静止消退的病理过程。下丘脑-垂体轴侵犯影像表现晚于临床。CT可显示LCH不同阶段的肺损害。结论　LCH各脏器损害的影像学表现缺乏特异性。好发部位的典型CT表现可提示诊断,同时对辅助LCH分型、确定治疗方案、估计预后有一定价值。     

18F-FDG PET/CT对骨朗格汉斯细胞组织细胞增生症的鉴别诊断

朗格汉斯细胞组织细胞增生症(LCH)原名组织细胞增生症X,是一组单克隆起源的树突状细胞增生性疾病,此组织细胞具有朗格汉斯细胞的特征,主要以骨质破坏、良性局灶性组织细胞增生和嗜酸性粒细胞浸润为特点。目前LCH发病原因不明,可见于任何年龄,但50%以上病例发生于1~15岁的儿童,可累及多系统及多器官,起病情况不一,严重者可出现广泛的脏器浸润伴发热和体重减轻。以单纯骨破坏为表现的LCH易被误诊,因此笔者选取2例LCH病例,通过对其18F-FDG PET/CT的影像学特征及临床资料进行鉴别诊断,归纳总结诊断思路,以提高影像诊断的准确率。     

Integrated imaging of systemic Langerhans cell histiocytosis in an infant

Langerhans cell histiocytosis (LCH) is a myeloid neoplasm characterized by a clonal proliferation of CD1a+/CD207+ dendritic cells. Although individuals of any age can be affected, the disease is most common in infants younger than 5 years of age, especially males. A wide range of manifestations, from asymptomatic to aggressive, have been described, along with multiorgan involvement. Even though the majority of bone lesions are observed, skin, lymph nodes, brain and lungs can also be involved. The involvement of hematopoietic system, including bone marrow, liver and spleen, is less frequent yet associated with worse prognosis, due to a worse treatment response. Diagnosis of LCH is based on the integration of clinical, laboratory, and radiological data; however, only histopathological examination might confirm it. As far as the spleen involvement is concerned, according to literature, it has been reported in about 15% patients with multisystem involvement, nonetheless only a few cases show parenchymal lesions. The present study reports the case of an infant with LCH with multisystem involvement, including bone, skin, liver, and spleen, with evidence of parenchymal lesions.     

Chronic recurrent multifocal osteomyelitis involving the mandible: case reports and review of the literature

Chronic recurrent multifocal osteomyelitis (CRMO) is an aseptic inflammatory disorder of unknown cause occurring in children and adolescents. It is characterized by multifocal bone lesions with pain and swelling recurring over months to years. Lesions usually involve the metaphyses of the long bones and involvement of the jaw is rare. The clinical presentation, radiographic appearance and histology of a case of CRMO involving the mandible in an 8-year-old girl are documented. The radiographic appearance of another three cases is also described.     

Lacrimal gland and perioptic nerve lesions due to Langerhans cell histiocytosis (2007: 9b)

We report a patient presenting with bilateral lacrimal gland involvement and perioptic nerve sheath lesions due to Langerhans cell histiocytosis (LCH) invasion. LCH is a rare multisystemic disease characterized by a clonal proliferation of Langerhans cells. All organs may be involved with a clinical spectrum ranging from a solitary bone lesion to a severe life-threatening multisystem disease. Osteolytic orbital bone lesions with extension into the adjacent orbital soft tissues have been described. To our knowledge, lacrimal gland involvement has probably been described only once before. Perioptic nerve lesions are also very rare, having been described only three times before. Precisely correct answers were received by closing date from: Kemal DEMIR, Istanbul, Turkey Jose Carlos GALLEGO, Ferrol, Spain Manabu MINAMI, Tsukuba, Japan Ram PRAKASH GALWA, Chandigarh, India Annemie SNOECKX, Zandhoven, Belgium Panagiotis TSIRKINIDIS, Athens, Greece Filip VANHOENACKER, Duffel, Belgium     

MR imaging of infiltrative muscle involvement with Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) consists of three clinical syndromes of varying severity resulting from basic cellular defect leading to lipid deposition within histiocytes. Radiologically, bone lesions are similar in all three forms of LCH and are due to bone destruction. An eosinophilic granuloma of the bone can involve skeletal muscle by direct extension from the bone. However, skeletal muscle involvement is rare and is not reported on MR imaging previously in the English literature. Our case not only shows biopsy-proven muscle involvement by LCH but also reports the first diffuse nodular pattern of muscle involvement by LCH.     

Spontaneous regression of supratentorial intracerebral Langerhans' cell histiocytosis

Langerhans' cell histiocytosis (LCH) describes a group of conditions affecting the reticuloendothelial system. It includes the subtypes of Letterer-Siwe disease, Hand-Schuller-Christian disease and eosinophilic granuloma and most often presents in childhood. Intracranial involvement in LCH is usually restricted to the hypothalamic-pituitary axis or involves extra-axial extension from skull vault lesions. Supratentorial intracerebral lesions with mass effect and enhancement have rarely been described and are not included in the magnetic resonance based classification system of neurological LCH. We present the MRI of a patient with multisystem LCH with spontaneous resolution of enhancing temporal lobe lesions.     

Histiocytosis X: radiologic evaluation of 52 patients

Fifty-two patients with histologically proven histiocytosis X and intrathoracic and/or bone lesions have been evaluated. In the group, bone lesions appear clearly prevalent (51/52 patients) mainly as localized monostotic disease (31/51). Flat bones were more frequently involved, with special evidence for skull and ribs. Short as well as long bones were only seldom compromised, almost only in polyostotic and generalized disease. On the basis of our experience the most suggestive radiological findings in bone to diagnose an histiocytosis X were the following: preferential involvement of a flat bone, osteolytic pattern, sharp borders (72.9%), rare evidence of pathological fracture (20%) and periostitis (10%). On the contrary, the involvement of the neighbouring soft tissues (38.8%) appears to be significant.     

郎格罕细胞组织细胞增生症的临床影像学分析

目的探讨组织细胞增生症(LCH)的临床影像学特点。方法回顾性分析11例临床证实的LCH,X线检查11例,CT检查1例,MRI检查1例。结果肺部弥漫网点状阴影6例;肝脾肿大6例。骨骼病变中:虫噬样破坏3例,囊样膨胀性改变3例,广泛溶骨性破坏5例;软组织肿块4例;骨膜反应5例。MRI检查1例,骨骼病变呈稍长T1、长T2信号。结论LCH骨骼影像学表现具有特征性:累及长管骨时,多有骨膜反应;累及脊椎骨时,椎间软骨多不受累;所有骨骼病变均可出现软组织肿块。LCH单系统受累患者临床预后较好。     

儿童组织细胞增生症X颅骨病变的影象学诊断

分析儿童组织细胞增生症X颅骨病变的影像学表现和特点，并结合病理阐述其在临床诊断中的价值。材料和方法：回顾性分析经病理证实的儿童组织细胞增生症X伴颅骨破坏者21例，其中男性16例，女性5例。年龄从8个月～9岁，平均3．8岁。除X线平片外，13例还进行了CT扫描，其中单纯平扫5例，平扫＋增强8例。结果：21例颅骨破坏中，颅顶部破坏20例，占95％；颅底部9例，占42．9％；眼眶7例，占33．3％；面颅骨3例，占143％。结论：本症颅骨病变在X线影像学上有较特殊的表现，是临床诊断和鉴别诊断的重要手段，有时甚至可起到决定性的作用，并能有助于观察疗效，了解有无复发等。     

Imaging of multisystem Langerhans cell histiocytosis in an adult

Langerhans cell histiocytosis (LCH) is a rare disease of uncertain aetiology and is well recognised in children. LCH is characterized by granulomatous infiltration of the lung, bone, skin, lymph nodes, liver, spleen, brain, kidneys, and endocrine system. Manifestations vary depending on whether there is single or multisystem involvement. Clinical symptoms are often non-specific and imaging often plays a vital role in suggesting the diagnosis.We will illustrate and describe the imaging findings of multisystem LCH in an adult patient affecting the lung, liver, thyroid, lymph nodes, suprasellar region, and possibly pancreas. Increased familiarity with the imaging features of multisystem LCH should lead to increased diagnostic confidence and potentially earlier disease diagnosis.     

Langerhans' cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up

OBJECTIVE: The purpose of this study was to review the initial clinical and radiologic manifestations and the follow-up of pediatric patients with Langerhans' cell histiocytosis affecting the temporal bone. MATERIALS AND METHODS: We retrospectively studied 14 patients with Langerhans' cell histiocytosis affecting the temporal bone. All patients were examined initially and sequentially with CT. In six patients, MR imaging was also done. RESULTS: Temporal bone involvement was the initial form of presentation in 12 patients. In eight patients, temporal bone involvement presented as an isolated manifestation, and in four it was associated with multisystemic involvement. In the remaining two patients, temporal bone involvement appeared during the course of the Langerhans' cell histiocytosis. Bilateral involvement was seen in four patients. In two patients, the temporal bone was affected only at the petrous apex. CT showed destruction of bone in all 14 patients and an associated soft-tissue homogeneous mass after injection of i.v. contrast material in 12 patients. CT showed a heterogeneous appearance of the soft-tissue mass in two patients. The average period of follow-up was 5 years. In seven of the 14 patients, the disease had a satisfactory evolution in which the bony lesions of the temporal bone reossified and remodeled over the course of a year. CONCLUSION: In Langerhans' cell histiocytosis, involvement of the temporal bone is usually seen on radiographs as extensive lytic lesions associated with soft-tissue masses. The lesions that remit show early disappearance of the soft-tissue mass, followed by reossification and remodeling of the involved bone. Patients with limited initial involvement of the temporal bone have a better prognosis on long-term follow-up than do patients with the multisystemic form.     

MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis

BACKGROUND AND PURPOSE: Intracranial manifestations of Langerhans cell histiocytosis (LCH) are underestimated in frequency and diversity. We categorized the spectrum of MR imaging changes in LCH. METHODS: We retrospectively reviewed 474 MR images in 163 patients with LCH and 55 control subjects. Lesions were characterized by anatomic region and signal intensity. Brain atrophy was assessed. RESULTS: We noted osseous lesions in the craniofacial or skull bones in 56% of patients, meningeal lesions in 29%, and choroid-plexus involvement in 6%. In the hypothalamic-pituitary region, infundibular thickening occurred in 50%; pronounced hypothalamic mass lesions in 10%; and infundibular atrophy in 29%. The pineal gland had a cystic appearance in 28%, and pineal-gland enlargement (>10 mm) was noted in 14%. Nonspecific paranasal-sinus or mastoid opacifications were seen in 55% of patients versus 20% of controls, and accentuated Virchow-Robin spaces occurred in 70% of patients versus 27% of controls (P <.001). Intra-axial, white-matter parenchymal changes resulted in a leukoencephalopathy-like pattern in 36%. Enhancing lesions in a vascular distribution were noted in 5%. Gray-matter changes suggestive of neurodegeneration were identified in the cerebellar dentate nucleus in 40% and in the supratentorial basal ganglia in 26%. All patients with neurodegenerative lesions had lesions in the extra-axial spaces. Cerebral atrophy was found in 8%. CONCLUSION: In LCH, cranial and intracranial changes at MR imaging include 1) lesions of the craniofacial bone and skull base with or without soft-tissue extension; 2) intracranial, extra-axial changes (hypothalamic-pituitary region, meninges, circumventricular organs); 3) intracranial, intra-axial changes (white matter and gray matter); and 4) cerebral atrophy.     

40例儿童郎罕细胞组织细胞病骨骼病变的影像学诊断

目的 :探讨儿童郎罕细胞组织细胞病 (LCH)骨骼病变的影像学表现及其诊断价值。材料和方法 :回顾性分析经病理证实的儿童LCH 40例 ,均行X线平片检查 ,其中 8例CT扫描。 14例分别于 1个月～ 2年内随访。结果 :本组中颅骨破坏 32例 ,占 80 %;脊柱破坏 9例 ,占 2 2 .5 %;长骨破坏 13例 ,占 32 .5 %;扁骨及不规则骨破坏 4例 ,占 10 %。嗜酸性肉芽肿以颅骨、长骨和脊柱多发 ,其他类型以颅骨多发。结论 :LCH骨骼病变具有多发性、多样性和多变性的特点 ;X线平片是本症诊断的基本手段 ,CT对隐蔽部位病变及病变细微结构的显示优于平片。     

Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients

OBJECTIVE: The purpose of this study was to review the craniofacial and intracranial clinical and radiologic manifestations of patients diagnosed with Langerhans cell histiocytosis (LCH). This report will compare the frequency of the various manifestations found in our series with those reported in the medical literature. CONCLUSION: In LCH, involvement of the calvaria, skull base, maxillofacial bones, and hypothalamic-pituitary axis is fairly common. The precise location of these lesions contributes to the variety of clinical manifestations of LCH, which includes scalp and/or facial swelling, seizures, hearing loss, recurrent otitis media, gingival bleeding, proptosis, diabetes insipidus, and cranial nerve palsies.     

Multisystem Langerhans cell histiocytosis: Literature review and case report

Langerhans cell histiocytosis (LCH) refers to a group of diseases of unknown etiology, typically discovered in childhood, characterized by the accumulation of Langerhans cells (white blood cells with large cell nuclei that may contain cytoplasmic histiocytosis X bodies) involving one or more organ systems, including bones, lungs, pituitary gland, skin, lymph nodes, and liver. This disease is also known as histiocytosis X or eosinophilic granuloma. Pulmonary LCH is common (identified in 40% of LCH patients) and may be isolated to the lung or involve other organs. Although LCH is characterized by clonal cell proliferation, adult LCH is considered likely to represent the manifestation of an aberrant immune response to an unspecified antigenic stimulus rather than a manifestation of tumor proliferation. We report a very complicated clinical case of LCH, with multiple organ damage that received a variety of different diagnoses. An LCH diagnosis was confirmed based on postoperative spinal cord pathology results and immunohistochemistry examinations. This case report highlights the clinical, laboratory, and imaging signs observed in this case that should be noted to help doctors more quickly recognize, diagnose, and treat similar cases.