Imaging findings in seven cases of congenital infantile myofibromatosis with cerebral, spinal, or head and neck involvement

Introduction

Congenital infantile myofibromatosis (IM) is a rare mesenchymal disease, presenting with tumors in the skin, muscle, viscera, bone, and subcutaneous tissue. It can present as (a) a solitary form with subcutaneous, erythematous nodules, (b) a multicentric form with subcutaneous, muscle, and/or bony lesions, and (c) a multicentric form with visceral involvement. Cerebral or spinal involvement in myofibromatosis has been reported rarely.

Methods

We report seven cases of histology-proven infantile myofibromatosis with brain, spine, and/or head and neck involvement.

Results

In three patients with multiple subcutaneous nodules, a multicentric form of IM with visceral involvement was diagnosed. In three patients, a multicentric form without visceral involvement was found. Two patients had brain involvement, and four patients had vertebral body involvement.

Conclusion

In a newborn presenting with intraparenchymal brain lesions, epidural spinal masses, and/or vertebra plana or lytic lesions of the calvarium and spine, infantile myofibromatosis should be considered as a possible differential diagnosis. The presence of subcutaneous or muscular nodules facilitates the diagnosis.     

Arterial manifestations of Beh?et disease

Beh?et disease is an uncommon systemic process that appears most often in the third or fourth decade of life. It is characterized by recurrent orogenital ulcers and ocular and cutaneous inflammatory lesions. Cardiovascular involvement, which may be arterial or venous, is rare but carries a particularly poor prognosis. Three new cases are reported, and they are characterized by typical orogenital and cutaneous manifestations and associated severe vasculitis. The arterial findings in these cases were occlusions and aneurysms and pseudoaneurysms of the aorta and pulmonary, brachiocephalic, and visceral arteries. From these cases and those previously reported, arterial involvement in Beh?et disease can be characterized by saccular aneurysms or occlusions of multiple large vessels in young adults.     

Intravascular malignant lymphomatosis

Intravascular malignant lymphomatosis is a rare and probably often overlooked disease characterised by massive intravascular proliferation of lymphoid cells, usually with a poor prognosis. CT and MRI appearances are nonspecific; the most suggestive finding being both asymmetrical, bilateral, contrast enhancing high-signal areas on T2 weighting and infarct-like lesions of the cortex and basal ganglia. We report two patients with previously unreported dural and spinal cord involvement.     

Retrocecal mucus-secreting tumors of the appendix (mucoceles). Apropos of 3 radiologically diagnosed cases

The term of appendiceal mucocele has been applied to a group of lesions distending the lumen of the appendix; filled with mucus. About 15% of these lesions are malignant tumors (cystadenocarcinomas). All these lesions may lead to pseudomyxoma peritonei, but only the peritoneal involvement due to a malignant tumor (adenocarcinoma) has multiple recurrences and poor prognosis. Benign lesions are cured by appendicectomy, even if a peritoneal "gelatinous" effusion is present. These lesions can usually be early diagnosed by US and CT features if the mucocele extends behind the cecum in the retroperitoneal space, especially if the wall is calcified. A colic examination (with barium enema or colonoscopy) must be taken event if the diagnosis is made by US or CT means, because of the significant association of mucocele with other colonic tumors.     

Blunt abdominal trauma in adults: role of CT in the diagnosis and management of visceral injuries Part 2: Gastrointestinal tract and retroperitoneal organs

Computed tomography plays an important role in the detection and management of blunt visceral injuries in adults. Current standard examination techniques enable detection of the majority of perforating or devascularizing bowel injuries, although diagnostic findings are often subtle and meticulous inspection is required. Computed tomography may demonstrate pancreatic contusions and lacerations and help in distinguishing minor traumatic lesions without involvement of the pancreatic duct (organ injury scale, grades I and II) from deep lacerations with ductal involvement (grades III and V). Computed tomography enables distinguishing renal contusions and minor cortical lacerations that can usually be managed conservatively (injuries of grades I–III) from corticomedullary lacerations and injuries of the major renal vessels (grades IV and V) that have a less favorable prognosis and more commonly require surgical repair. In addition, CT is well suited for the detection of active renal hemorrhage and guidance of transcatheter embolization treatment and delineation of preexisting benign or malignant pathologies that may predispose to posttraumatic hemorrhage. The radiologist's awareness of the diagnostic CT findings of abdominal visceral injuries as well as their clinical and surgical implications are important prerequisites for optimal patient management. Received 22 July 1997; Revision received 16 October 1997; Accepted 23 October 1997     

Brain involvement in Hodgkin's disease: case reports and review of the literature

Hodgkin's disease involving in the central nervous system is extremely rare. It usually spreads contiguously, as visceral involvement is generally thought to occur secondary to involved adjacent lymph nodes. We report three such cases found in our institution in the last two decades. Based upon our limited experience together with reported data, whole brain irradiation combined with systemic chemotherapy remains the treatment of choice for these lesions.     

Infantile myofibromatosis

 Infantile myofibromatosis is a mesenchymal tumor most commonly seen in infancy. The tumors have a variable appearance on CT/MR and often simulate a more aggressive neoplasm. This report describes CT/MR findings in cases of infantile myofibromatosis with pathologic correlation. Discussion into the success of imaging in suggesting the correct diagnosis is also addressed. Infantile myofibromatosis is a mesenchymal disorder of infancy characterized by the presence of tumorous nodules in the skin, subcutaneous tissue, muscle, viscera, and bone. Cases of solitary and multiple lesions have been described. We present the clinical, histologic, and radiographic findings of one case of the solitary form of infantile myofibromatosis that was recently diagnosed at our hospital.     

Bony metastases of the fingers. Apropos of 3 cases. Review of the literature

Bone metastases of fingers are rare, are of highest incidence in men 40 to 60 years, and may reveal the presence of a primary cancer. Radiologic imaging shows almost constant osteolytic lesions, the primary usually being located in the bronchi. Short-term prognosis is poor, with a mean survival of three and a half months. Bone biopsy should be conducted routinely to determine nature of lesion in fingers.     

Langerhans cell histiocytosis of bone.

Langerhans cell histiocytosis (LCH), previously called histiocytosis X, refers to a spectrum of disease characterized by idiopathic proliferation of histiocytes producing focal or systemic manifestations. Causes and pathogenesis remain unclear. However, recent studies suggest abnormal immune regulation as an important factor. The three classic syndromes may have considerable clinical overlap: eosinophilic granuloma, in which the disease is limited to bone in patients usually 5-15 years old; Hand-Schüller-Christian disease, characterized by multifocal bone lesions and extraskeletal involvement of the reticuloendothelial system (RES) usually seen in children 1-5 years old; and Letterer-Siwe disease, in which there is disseminated involvement of the RES with a fulminant clinical course in children less than 2 years old. Osseous involvement is typically in the flat bones, with lesions of the skull, pelvis, and ribs accounting for more than half of all lesions. About 30% of lesions are in long bones. Radiographic appearance of osseous LCH depends on site of involvement and phase of the disease. Early lesions appear aggressive with poorly defined margins and lamellated periosteal reaction. Late lesions appear well defined and may show sclerotic margins and expanded remodeled appearance.     

Roentgenographic aspects of non-Hodgkin's lymphomas presenting with osseous lesions

Radiographs of 992 patients with previously untreated non-Hodgkin's lymphoma were reviewed, and bone involvement was found in 61. Ten patients had primary lymphoma of bone and 51 patients had concomitant lymph node and/or visceral involvement or several affected bones. Roentgenographic analysis of all the bone lesions showed that osteolysis predominated, but without specific diagnostic features, and that cortical destruction and soft tissue involvement carry an adverse prognosis. Routine skeletal X-ray survey in the initial staging of non-Hodgkin's lymphomas is essential.     

Diffuse skeletal angiomatosis

Diffuse skeletal angiomatosis is a rare disease that is usually associated with visceral angiomatosis. The bony changes are striking and the roentgen examination is the most important diagnostic measure. The lesions are almost invariably lytic and are usually well-defined. The most commonly involved bones are the femur, the pelvis, ribs, humerus, the skull, and vertebrae. The pattern of bony involvement is reviewed and the types of involvement are illustrated including a unique example with extensive changes in the bones of the hand.     

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.     

Akute disseminierte Enzephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is an acute widespread autoimmune demyelinating condition, which principally affects the white matter of the brain and spinal cord. It usually follows an infection or vaccination. The typical presentation is that of multifocal neurologic disturbances accompanied by change in mental status. CSF analysis reveals lymphocytic pleocytosis and elevated protein content, but may also yield normal results. MRI is regarded as the diagnostic imaging modality of choice and typically demonstrates involvement of deep cerebral hemispheric and subcortical white matter as well as lesions in the basal ganglia, gray-white junction, diencephalon, brainstem, cerebellum and spinal cord. Unlike multiple sclerosis (MS), ADEM has a monophasic course and a favorable long-term prognosis.     

<Superscript>68</Superscript>Ga-DOTA-Affibody molecule for in vivo assessment of HER2/neu expression with PET

Purpose  

Overexpression of HER2/neu in breast cancer is correlated with a poor prognosis. It may vary between primary tumors and metastatic lesions and change during the treatment. Therefore, there is a need for a new means to assess HER2/neu expression in vivo. In this work, we used ⁶⁸Ga-labeled DOTA-Z_HER2:2891-Affibody to monitor HER2/neu expression in a panel of breast cancer xenografts.     

Variation of usual interstitial pneumonia using HRCT in Scleroderma Patients

Progressive Systemic Sclerosis or Scleroderma is a systemic inflammation marked by endothelial damaged, fibrosis, and inflammation in skin, joints, and visceral organs. Pulmonary hypertension and interstitial pulmonary disease (Ssc-ILD) are the most reported pulmonary complications in scleroderma patients. The pathogenesis of SSc- ILD is not well understood and the spectrum of SSc-ILD ranges from minimal lung involvement, which is often non-progressive, to severe illnesses. Usual Interstitial Pneumonia''s (UIP) is one pattern of Ssc-ILD, marked in one-third of the patients with characteristic of honeycomb appearance and bronchiectasis. It is very important to determine the UIP based on radiology imaging especially with the presence of a poor prognosis in patients with UIP. This case report will discuss the importance of finding UIP-type ILD patterns based on HRCT in patients with scleroderma and different outcome.     

Fluorine-18 deoxyglucose positron emission tomography for the detection of bone metastases in patients with non-small cell lung cancer

Despite advances in morphological imaging, some patients with lung cancer are found to have non resectable disease at surgery or die of recurrence within a year of surgery. At present, metastatic bone involvement is usually assessed using bone scintigraphy, which has a high sensitivity but a poor specificity. We have attempted to evaluate the utility of the fluorine-18 deoxyglucose positron emission tomography (FDG PET) for the detection of bone metastasis. One hundred and ten consecutive patients with histological diagnosis of non-small cell lung cancer (NSCLC) who underwent both FDG PET and bone scintigraphy were selected for this review. In this group, there were 43 patients with metastatic disease (stage IV). Among these, 21 (19% of total group) had one or several bone metastases confirmed by biopsy (n = 8) or radiographic techniques (n = 13). Radionuclide bone scanning correctly identified 54 out of 89 cases without osseous involvement and 19 out of 21 osseous involvements. On the other hand, FDG PET correctly identified the absence of osseous involvement in 87 out of 89 patients and the presence of bone metastasis in 19 out of 21 patients. Thus using PET there were two false-negative and two false-positive cases. PET and bone scanning had, respectively, an accuracy of 96% and 66% in the evaluation of osseous involvement in patients with NSCLC. In conclusion, our data suggest that whole-body FDG PET may be useful in detecting bone metastases in patients with known NSCLC. Received 10 March and in revised form 7 May 1998     

脏层胸膜受侵对T2非小细胞肺癌预后的影响

目的评估T2非小细胞肺癌（non-small cell lung cancer,NSCLC）中脏层胸膜受侵（visceral pleural invasion,VPI）与其他临床病理特征的关系,评价其对预后的价值。方法行根治性手术切除的T2非小细胞肺癌的患者628例,其中ⅠB期（T2N0M0）265例,ⅡB期（T2N1M0）223例和ⅢA期（T2N2M0）140例。根据是否有VPI分成两组（Ⅰ组无受侵,Ⅱ组有受侵）。对两组病例肿瘤大小、组织学分类、是否有淋巴结的转移和生存率进行比较。结果Ⅱ组患者共有136例,占所有患者的21.7%,其中肿瘤直径≤3 cm 70例,肿瘤直径〉3 cm 66例。第Ⅰ组患者5年和10年生存率分别为53.7%和40.0%,而第Ⅱ组患者分别为25.7%和11.0%（P〈0.01）。IB期患者的生存率因肿瘤直径的不同而有显著不同,当考虑是否存在VPI时,这种差异更加显著。ⅡB和ⅢA期的患者不论肿瘤直径大小,两组患者的生存率差异无统计学意义。与其他组织型患者相比,腺癌患者更易出现VPI（38.4%）,VPI的患者更易出现淋巴结转移（14.7%N0,23.3%N13,2.1%N2;N2和N0相比较,P〈0.01;N2与N1比较,P〈0.01）。结论 VPI为T2非小细胞肺癌预后差的一项因素,它与更广泛的淋巴结转移和较低的生存率直接相关。     

Imaging of ischemic heart disease

Despite advances in the understanding and treatment of ischemic cardiomyopathy, characterized by extensive coronary artery disease and left ventricular (LV) dysfunction, the prognosis remains poor with only a 50-60% 5-year survival rate. The composition of atherosclerotic lesions is currently regarded as being more important than the degree of stenosis in determining acute events. If imaging techniques could distinguish vulnerable from stable plaques, then high-risk patient subgroups could be identified. Another important concept is that LV dysfunction may be the result of either scarring due to necrosis or to the presence of myocardial hibernation, in which there is sufficient blood flow to sustain viable myocytes, but insufficient to maintain systolic contraction. This concept of myocardial viability is critical for making optimal clinical management decisions. This review describes how noninvasive imaging methods can be used to distinguish regions of irreversibly injured myocardium from viable but hibernating segments. Technical advances in CT and MR have made imaging of the beating heart possible. Considerable clinical progress has already been made and further cardiac applications are expected. Radiologists therefore have new opportunities for involvement in cardiac imaging but must recognize the political implications as well as the diagnostic potential of these modalities not only for the heart, but also for the whole vascular system. This review focuses on imaging myocardial injury. It compares state-of-the-art CT and MR with more established yet contemporary echocardiography and nuclear scintigraphy.     

小儿流行性乙型脑炎磁共振波谱特征及随访研究

目的：探讨磁共振氢质子波谱（1H-MRS）对小儿流行性乙型脑炎预后评价的价值。方法：对17例常规MRI有信号异常的乙脑患儿行1H-MRS扫描,并选择15例同龄健康志愿者对照。病例组在3个月内进行随访并复查MRI,根据随访结果将患者分治愈组和未治愈组,回顾性分析两组患儿首次1H-MRS特征。结果：病灶在T1WI呈斑片状等或稍低信号、T2WI及FLAIR呈稍高或高信号,其中累及灰质核团13例,均呈对称性分布,边界清晰。17例患儿中1H-MRS均见不同高度Lac峰,7例NAA峰有明显下降,2例Cho峰轻度降低,1例出现Cr峰下降,3例出现MI峰。病例组与对照组之间NAA/（Cr＋Cho）值差异有显著性意义（t=5.98,P〈0.05）。根据随访结果,治愈组11例,未治愈组6例,两组NAA/（Cho＋Cr）差异有显著性意义（t=3.45,P〈0.05）。结论：乙脑患儿病变易累及灰质核团,1H-MRS多见Lac双峰,NAA明显下降往往提示患儿病情较重,预后不良。1H-MRS能反映乙脑严重程度并判断预后,是常规MRI有益补充。     

Duktales Adenokarzinom und ungewöhnliche Differenzialdiagnosen

Ductal pancreatic adenocarcinoma is by far the most common solid tumor of the pancreas. It has a very poor prognosis, especially in the more advanced stages which are no longer locally confined. Due to mostly unspecific symptoms, imaging is key in the diagnostic process. Because of the widespread use of imaging techniques, incidental findings are to a greater extent discovered in the pancreas, which subsequently entail further work-up. Ductal pancreatic adenocarcinoma can be mimicked by a large number of different lesions, such as anatomical variants, peripancreatic structures and tumors, rarer primary solid pancreatic tumors, cystic tumors, metastases or different variants of pancreatitis. Additionally, a number of precursor lesions can be differentiated. The correct classification is thus important as an early diagnosis of ductal pancreatic adenocarcinoma is relevant for the prognosis and because the possibly avoidable treatment is very invasive. All major imaging techniques are principally suitable for pancreatic imaging. In addition to sonography of the abdomen, usually the baseline diagnostic tool, computed tomography (CT) with its superior spatial resolution, magnetic resonance imaging (MRI) with its good soft tissue differentiation capabilities, possibly in combination with MR cholangiopancreatography (MRCP), endosonography with its extraordinary spatial resolution, conceivably with additional endoscopic retrograde CP or the option of direct biopsy and finally positron emission tomography CT (PET-CT) as a molecular imaging tool are all particularly useful modalities. The various techniques all have its advantages and disadvantages; depending on the individual situation they may need to be combined.