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Peptide-based immunotherapy does not usually elicit strong immunological and clinical responses in patients with end-stage cancer, including sarcoma. Here we report a myxofibrosarcoma patient who showed a strong clinical response to peptide vaccinations and whose immune responses were reboosted by anti-PD1 therapy combined with peptide vaccinations. The 46-year-old man showed a strong response to the peptide vaccinations (papillomavirus binding factor peptide, survivin-2B peptide, incomplete Freund's adjuvant, and polyethylene glycol-conjugated interferon-alpha 2a) and subsequent wide necrosis and massive infiltration of CD8+ T cells in a recurrent tumor. The patient's immune responses weakened after surgical resection; however, they were reboosted following the administration of nivolumab combined with peptide vaccinations. Thus, anti-PD1 therapy combined with peptide vaccinations might be beneficial, as suggested by the observations in this sarcoma patient.

The aim of this study was to investigate the pathological classifications, clinical features, and natural history of pediatric cardiac tumors to provide a basis for the selection of an appropriate therapeutic method. The medical records of in- or outpatients with cardiac tumors at four hospitals were classified to analyze various types of tumor growth locations, clinical manifestations, surgical indications, and long-term follow-up results. There were 166 patients, including 158 with primary cardiac tumors, six with metastatic cardiac tumors, and two with unclassified cardiac tumors. Among the 158 cases of primary cardiac tumor, 150 were benign and eight were malignant. The rhabdomyoma, fibroma, and myxoma are the most common types of benign cardiac tumors. The major clinical manifestations of cardiac tumors include outflow tract obstruction, arrhythmia, dyspnea, pericardial effusion, heart failure, and seizures. Among the 59 patients who underwent surgery, 49 had primary benign cardiac tumors, eight had primary malignant tumors, and two had malignant metastatic tumors. Post-surgery, nine of the patients had residual tumor tissues that did not significantly affect their hemodynamics. Following surgery, there were two cases of recurrence and nine deaths, including four of benign and five of malignant tumors with mortality rates of 8.2 and 50.0 %, respectively. Of the remaining 107 cases of patients who did not undergo surgery, five (4.7 %) died., Conclusion: The primary benign cardiac tumors are the predominant pediatric cardiac tumors, of which rhabdomyoma, fibroma, and myxoma are the most common types. If severe symptoms are nonexistent and the hemodynamics is unaffected, most of the patients can survive in the long term despite the tumors. What is known: • Pediatric cardiac tumors are rare and are predominantly primary and benign. • The symptoms of heart failure, arrhythmia, and outflow obstruction are the most severe complications of cardiac tumors. What is new: • The rhabdomyoma, fibroma, and myxoma are the most common types of primary benign cardiac tumors. • If severe symptoms are not present and the hemodynamics are unaffected, most of the patients can survive in the long term despite the tumors.

MRI is a valuable noninvasive tool that helps in predicting the type of cardiac tumors and guiding management decisions. Several reports have described the appearance of cardiac fibromas on MRI, which typically show hyperenhancement on myocardial delayed enhancement (MDE) imaging, with or without a dark core. This report demonstrates the unique appearance of a large solitary ventricular septal cardiac fibroma in a 5-month-old patient on MDE imaging, with two discrete dark cores, each surrounded by a hyperenhancing pseudocapsule.

Patients with anal complaints require a high level of sensitivity of the involved healthcare professionals. A large variety of different pathological changes lead to very similar clinical symptoms. A carefully guided history interview is able to target in many cases the underlying condition. Treatment should not be performed before definite exclusion of any malignant disease. However, patients most of all blame hemorrhoids for any anal physical discomfort and visit the physician with high expectations in treatment of their supposed disease. The following summary ranks anal diseases by frequency considering their clinical appearance and delimitation and provides an overview of symptoms associated with hemorrhoids.

Objective: Myxofibrosarcoma (MFS) is characterized by a high frequency of local recurrence after surgery because of infiltrative growth of the tumor cells. This infiltrative growth creates a characteristic 'tail-like' pattern on magnetic resonance imaging (MRI), and it has been reported that this pattern is especially obvious on gadolinium-enhanced MRI (Gd MRI). However, the relationship between the tail-like pattern seen on Gd MRI and clinicopathological features of MFS is still not clear. In this study, we performed a retrospective analysis to identify clinicopathological factors related to the tail-like pattern of the MRI findings in patients with MFS., Materials and Methods: We retrospectively analyzed 50 patients with MFS to identify factors related to the tail-like pattern., Results: On Gd MRI, 32 of the 50 patients presented the tail-like pattern, whereas 18 presented a solid pattern. The clincopathological factors related to the tail-like pattern were evaluated by chi-squared test. A superficial origin (p = 0.0009) was most significantly related to the tail-like pattern. The 5-year recurrence-free survival (RFS) rate was 75.6 % for patients showing the tail-like pattern and 90.9 % for those showing the solid pattern. The corresponding 5-year disease-free survival (DFS) rates were 64.7 and 79.3 %, respectively. Thus in terms of both 5-year RFS and DFS, patients with the tail-like pattern tended to have a poorer outcome., Conclusion: A superficial origin of MFS is significantly related to a tail-like pattern on Gd MRI. The tail-like pattern is associated with poorer prognosis. Further studies of tumor depth and the tail-like pattern on Gd MRI are needed.

Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by basal cell carcinoma, skeletal abnormalities, benign tumors including ovarian fibroma, and various other phenotypic expressions. Ovarian fibromas in NBCCS before puberty are very rare. We report a 6-year-old prepubescent girl with NBCCS showing skeletal abnormalities, medulloblastoma, and ovarian fibromas. The patient was referred to our hospital owing to abdominal distension. On admission, a huge elastic hard tumor was palpable and computed tomography showed a huge tumor of the left ovary. We performed a left salpingo-oophorectomy and diagnosed the tumor as a benign fibroma. Further examination of the computed tomography images showed skeletal abnormalities. In addition, the patient had a history of medulloblastoma at the age of 4 years. Therefore, we diagnosed NBCCS. A genetic examination indicated a novel 1 bp deletion in exon 18 (c.3055delG). Sequence analysis of exon 18 using DNA from the ovarian tumor revealed a mutant allele (c.3055delG) dominant to the wild-type allele, thus suggesting loss of heterozygosity in the PTCH1 gene, which is known to be associated with NBCCS. Conclusion On the basis of our experience, physicians treating pediatric ovarian tumors should be aware that such huge benign ovarian tumors may be a phenotype of NBCCS, as shown in our patient. In addition, genetic examination focusing on the PTCH1 gene might be important for diagnosis of NBCCS in pediatric patients.

Chondromyxoid fibroma (CMF) is a rare benign cartilage congener tumor comprising less than 1 % of primary bone tumors. Although the age range is wide, it is most commonly seen in the second and third decades. The most frequent location of CMF is in the long tubular bones of the lower extremities, particularly the proximal tibia and distal femur. Although the majority of chondromyxoid fibromas present as intramedullary tumors, a subgroup of chondromyxoid fibromas arising as surface lesions of the bone has recently been described. These are associated with an older mean age and an increased incidence of matrix calcifications. Chondromyxoid fibromas are rare in the distal ulna. We report a CMF presenting as a surface lesion of the distal metaphysis of the left ulna in a 41-year-old woman. We reviewed the literature on chondromyxoid fibromas involving the ulna and found that out of 22 cases, 1 was in the distal ulna, 13 in the proximal ulna, and in the remaining 8 the ulnar sites were unspecified. No case of chondromyxoid fibroma in the published literature had been designated as a surface lesion. Our own unpublished data include 70 chondromyxoid fibromas, 4 of which are in the ulna. Two of these are in the distal portion.

Intramuscular myxoma (IM) is a benign intramuscular neoplasm composed of fibroblasts and abundant myxoid stroma. Some malignant soft tissue tumors can undergo myxomatous degeneration, which makes it difficult to distinguish them from IM. We describe a case of IM of the buttock region mimicking low-grade fibromyxoid sarcoma. The tumor appeared as a well-defined ovoid mass with a cystic lesion on MRI images, and mild uptake on PET images was seen. This was originally misdiagnosed as low-grade fibromyxoid sarcoma (LGFMS) after core-needle biopsy. The mass was excised en bloc and sent for histology. The surgical specimen showed the features of LGFMS with the same characteristics as those mentioned in the previous biopsy report. After surgery, MUC4 expression, a highly sensitive and specific immunohistochemical marker for LGFMS, and FUS gene rearrangement by FISH was not detected upon re-examination; therefore, a conclusive diagnosis of IM was made. The patient had no local recurrence at the 3-year follow-up. Our case suggests that IM with mild FDG uptake is frequently confused with other low-grade malignant myxoid tumors. In addition, absence of MUC4 expression is the definitive key to distinguish IM from LGFMS.

Objective: To determine common imaging features of low-grade fibromyxoid sarcoma (LGFMS), a rare subtype of fibrosarcoma with a benign histological appearance but with a propensity for local recurrence and metastasis., Materials and Methods: By review of medical records, 29 patients (14 males, 15 females) with mean age of 41 years (range, 11-64 years) with diagnosis of LGFMS and imaging available in PACS were identified. The study included radiography (9/29), ultrasound (1/29), CT (14/29), and MRI (22/29). Imaging features evaluated included anatomic site, and lesion size, number, location, US echogenicity, CT attenuation, calcifications, MR signal intensity pattern, and contrast enhancement., Results: Twenty-two patients had imaging at initial diagnosis, and seven had imaging only at local recurrence. The majority of tumors (27/29) were deep in tissue, with mean size of 6.2 cm (range, 1.4-19.0 cm). Tumor was single at initial diagnosis and multiple at local recurrence; lower extremity was the most common site. On radiographs, tumor was visible in four of nine without bone erosion or calcification. Areas of CT attenuation hypodense to muscle were seen in 13/14 patients. Calcification was uncommon (3/14). On fluid-sensitive MRI sequences, the predominant signal intensity was hyperintense to muscle in 11/21 patients. In 13/21 patients, tumor displayed either a brain gyriform pattern of alternating hypointense and hyperintense signal intensity (9/13) or intralesional nodules (3/13); 1 case had both patterns. On US tumor was solid with heterogeneous echogenicity., Conclusion: LGFMS is commonly single at initial diagnosis and multiple at local recurrence. LGFMS frequently shows areas of CT attenuation hypodense to muscle and gyriform patterns of signal intensity and contrast enhancement at MRI.

Benign cartilage tumours of bone are the most common benign primary bone tumours and include osteochondroma, (en)chondroma, periosteal chondroma, chondroblastoma and chondromyxoid fibroma. These neoplasms often demonstrate typical imaging features, which in conjunction with lesion location and clinical history, often allow an accurate diagnosis. The aim of this article is to review the clinical and imaging features of benign cartilage neoplasms of bone, as well as the complications of these lesions.

Objective: The purpose of this study was to assess retrospectively the characteristics of FDG uptake in elastofibroma dorsi using integrated PET/CT., Methods: From 10,261 oncology FDG-PET/CT scans performed over a 2-year period, findings suggestive of elastofiboma dorsi were observed in 46 FDG-PET/CT scans of 34 patients. As 20 patients had bilateral lesions and 14 had unilateral lesions, a total of 75 elastofibroma dorsi lesions on images were identified in this study. For visual analysis of intensity of FDG uptake , a four-point grading system was used: grade 0 for no uptake, grade 1 for less uptake than the liver, grade 2 for uptake comparable to the liver, and grade 3 for intense uptake greater than the liver. For quantitative analysis, the standardized uptake value (SUV) was calculated. The relationships between SUV and age, blood glucose level, lesion size, and related symptoms were also assessed., Results: Among the 75 lesions, 4 had an uptake grade of 0, 41 had grade 1, 25 had grade 2, and 5 had grade 3. The mean SUV (±SD) of the 75 lesions was 2.0 ± 0.63 (range 0-5.1). The Pearson correlation coefficient test indicated a weak positive correlation between SUV and lesion size and no correlation between SUV and either age or blood glucose level. The SUVs of patients with symptoms due to the disease and patients without symptoms were almost the same., Conclusion: Mild and moderate uptake of FDG is frequently observed in elastofibroma dorsi, which should not be misinterpreted as abnormal accumulation observed in malignant lesions.

The term infantile digital fibromatosis describes a benign tumor of the group of fibromatoses. The prevalence rate is approximately 2.5% of all fibromatoses. The etiopathogenesis of infantile digital fibromatosis is unknown. These tumors can appear already at birth, but usually manifest during the first 3 years of life as solid, pink or skin-colored asymptomatic nodules on one or several fingers or toes. Because of the postoperative recurrence rate of 50-75% and the possibility of spontaneous regression, excision is not recommended according to currently available evidence.

A 5-year-old boy presented with acute onset of chorea and dysarthria. During his workup, a heart murmur was noted. Echocardiography revealed multiple mobile masses in the left ventricular outflow tract related to the mitral valve. Brain magnetic resonance imaging and magnetic resonance angiography did not detect any abnormalities. He underwent a subtotal resection of the mass to preserve valvular function. The anatomic pathology was papillary fibroelastoma.

Juvenile hyaline fibromatosis is a rare autosomal recessive disease of the connective tissue. We present the case of a 6-year-old normal mental developed boy with confluent pearly papules behind the ears and in the paranasal folds, firm nodules of the scalp, the back and metaphalangs, and severe gingival hypertrophy.

Introduction: The histological features and image findings on computed tomography (CT) and magnetic resonance imaging (MRI) for ovarian cystadenofibroma mimicking malignant ovarian tumor are reported., Case: A 62-year-old woman was diagnosed as an ovarian cancer on abdominal CT. However, it was diagnosed as a cystadenofibroma on magnetic resonance T2-weighted images that showed the solid components of the tumor with very low intensity. Although ovarian cystadenofibroma is a relatively rare benign tumor, its ultrasonographic feature of cystic lesions with solid components resembles that of malignant ovarian tumor., Conclusion: MRI was considered to be useful modality for ovarian cystadenofibroma to be effectively diagnosed preoperatively.

We report a rare case of fibroma of the tendon sheath arising from the fat pad in the knee. To our knowledge, only four previous cases of fibroma of the tendon sheath in the knee have been reported in the English literature. In our case, magnetic resonance imaging showed low signal intensity in the mass on T1- and T2-weighted images. The mass was excised by mini arthrotomy.

We report on the clinical, imaging-including ultrasound, computed tomography, and magnetic resonance imaging-and histological features of a fibroma of the tendon sheath with mineralized chondroid and osseous metaplasia, presenting as a semimembranosus bursitis. The anatomical characteristics of the semimembranosus bursa are demonstrated by dissection in a cadaveric specimen and correlated with the imaging findings in our patient.

Chondromyxoid fibroma constitutes a rare benign tumor of the bones, which has a potential of regional enlargement towards the local tissues. An adult male patient at the age of 28 suffered a fracture of the lateral malleolus due to an eccentric, lytic, lobular lesion in the epiphysis, during a basketball match. The differential diagnosis included the chondroblastoma, the aneurysmal cyst and the chondrosarcoma. After the removal of the tumor with curettage, both with curette and with a high-speed burr, osseous cement was placed in the cavity of the lesion. The biopsy of the tumor confirmed the diagnosis of the chondromyxoid fibroma. This is the first unusual location of the tumor in the national bibliography. The patient after 5 years post-operatively does not show either clinical or radiological signs of regional recurrence of the tumor.

Superficial acral fibromyxoma (SAFM) is a rare soft tissue tumor most often located in the ungual region of the fingers and toes. This tumor was first described in 2001, and since then very few cases have been reported. We present the case of a 35-year-old male with a SAFM located in the toe, with involvement of the nail and erosion of the distal phalanx. The lesion was surgically removed, and the histopathological study confirmed the diagnosis of SAFM. The differential diagnosis must be established with other myxoid tumors and with those lesions showing a predilection for the distal portions of the limbs. After 2 years, the patient remains disease free, with no disability of any kind.

Cardiac tumors in children are rare. Although most are histologically benign, they can be associated with life-threatening arrhythmias and sudden death. We report a 7-year-old boy, with a first episode of symptomatic tachycardia, who was found to have a left ventricular (LV) fibroma. He had a normal echocardiogram prior to an electrophysiology study, which revealed a sustained monomorphic ventricular tachycardia and a radio-opacity near the LV apex. These findings prompted a cardiac MRI, which demonstrated a discrete mass on his LV apex and free wall. Our case emphasizes that structural heart disease should be aggressively pursued in children presenting with ventricular tachycardia.

Solitary fibrous tumors (SFT) are rare, mostly fibroblastic tumors usually situated in the pleura. Extrapleural manifestations have been described. However, the oral cavity is an uncommon localisation of this tumor. We report the very unusual case of an SFT affecting the tongue that could be removed completely because of its clear delineation. Intraoperative incisional biopsies were used to exclude malignancy. For definitive classification of the tumor, additional histopathologic examinations had to be carried out. Because SFT exhibit malignant behavior only in exceptional cases and their recurrence after complete removal has never been encountered, surgery can focus on the preservation of undisturbed function of the tongue.

The case of a 30-year-old woman with multiple loose bodies in the shoulder joint is presented. The patient complained of discomfort of her right shoulder. Conventional radiographs failed to reveal any abnormality of the shoulder. On MRI numerous loose bodies were detected that resembled synovial chondromatosis. Histologically, the loose bodies were composed of a benign fibroblastic lesion with diagnostic features of fibroma of tendon sheath, each surfaced by synoviocytes. The clinicopathologic features of intra-articular fibroma of tendon sheath are reviewed. We propose using the term "synovial fibroma" for tumors with histopathologic features of fibroma of tendon sheath arising in the synovium of joints.

Ten cases of histologically proven chondromyxoid fibroma (CMF) of the foot and ankle with a mean follow-up of 6.1 years were retrospectively reviewed using the Scottish Bone Tumour Registry. The patients' mean age was 19 years; there were six males and four females. The anatomical locations were five phalangeal, three metatarsal, one tarsal affecting body of os calcis and one distal tibial. The median delay in presentation was 4.5 months. The modes of presentation were pain only (n=4), painful lump (n=4) and painless lump (n=2). The typical radiological finding was an expansile, lobulated, cystic lesion. Cortical erosion was documented in 80% patients. In four cases, curettage alone was carried out, while five patients underwent curettage along with autogenous bone grafting. One patient with distal phalangeal CMF had a primary toe amputation. Two patients had recurrences 9 and 16 months after their initial curettage. Both of them were males with proximal phalangeal CMF, associated with cortical erosion. Foot and phalangeal CMF initially treated with curettage only should be closely followed up, as we observed a 20% recurrence rate within a 2-year period. Cases featuring cortical erosion require thorough curettage and may require autogenous bone grafting to prevent fracture.

Low-grade fibromyxoid sarcoma is a rare, slow-growing, yet metastasizing neoplasm characterized by histopathological fibrous/myxoid zonal alternations, which occasionally display an intralesional multinodular appearance. We report on the radiological features of two cases of low-grade fibromyxoid sarcoma. Case 1 was a large ovoid mass with a well-defined margin in the thigh, showing the characteristic multiple, intralesional variable-sized nodules on high-frequency ultrasound (US) and magnetic resonance imaging (MRI), some of the nodules showing a target-like appearance. Case 2 was a dumbbell-shaped chest wall mass showing invasive features without the intralesional multinodular appearance on MRI. The gross radiological appearances of our two cases were somewhat dissimilar, but they shared a pattern of low/high signal zonal distinction without hemorrhage or necrosis within the masses on MRI.

A 56-year-old post-menopausal woman presented with abdominal swelling and shortness of breath. An evaluation of abdomen and pelvis showed ascites and a solid mass. Serum CA-125 level was elevated (509 IU/ml). A computerized tomography-angiography was performed since the pulses of upper extremities could not be palpated. The patient was diagnosed to be suffering from Takayasu arteritis. The exploratory laparotomy revealed a fibroma of the ovary.

We compared the efficacy of echocardiography (ECHO) and magnetic resonance imaging (MRI) for evaluating intracardiac masses. Over an 8-yr period, 28 patients, 21 males, 7 females, 16 days-60 years of age (mean 25 years) with a suspected intracardiac mass on ECHO (transthoracic in all; transesophageal in 9) underwent an MRI examination. Five patients had a contrast-enhanced MRI. ECHO and MRI were compared with respect to their technical adequacy, ability to detect and suggest the likely etiology of the mass, and provide additional information (masses not seen with the other technique, inflow or outflow obstruction, and intramural component of an intracavitary mass). With MRI, the image morphology (including signal intensity changes on the various sequences) and extracardiac manifestations were also evaluated. The diagnosis was confirmed by histopathology in 18, surgical inspection in 4, by follow- up imaging on conservative management in 5, and by typical extracardiac manifestations of the disease in 1 patient. Fifteen (54%) patients had tumors (benign 12, malignant 3), 5 had a thrombus or hematoma, and 4 each had infective or vascular lesions. Thirty-four masses (13 in ventricle, 11 septal, 7 atrial, 2 on valve and 1 in pulmonary artery) were seen on MRI, 28 of which were detected by ECHO. Transthoracic ECHO (TTE) and MRI were technically optimal in 82% and 100% of cases, respectively. Nine patients needed an additional transesophageal ECHO (TEE). Overall, MRI showed a mass in all patients, whereas ECHO missed it in 2 cases. In cases with a mass on both modalities, MRI detected 4 additional masses not seen on ECHO. MRI suggested the etiology in 21 (75%) cases, while the same was possible with ECHO (TTE and TEE) in 8 (29%) cases. Intramural component, extension into the inflow or outflow, outflow tract obstruction, and associated pericardial or extracardiac masses were better depicted on MRI. We conclude that MRI is advantageous over a combination of TTE and TEE for the detection and complete morphological and functional evaluation (hemodynamic effects) of cardiac masses.

We report a case of elastofibroma that occurred in both thighs and subscapular regions. The MR signal intensity of the lesions was typical for elastofibroma. Although bilateral involvement of the thighs is exceptional, the presence of concomitant characteristic bilateral subscapular lesions was helpful in making the diagnosis of elastofibroma.

A 19-year-old man presented with an elastofibroma in his left shoulder joint. The patient had had limitation of motion in his left arm for 3 years, especially when rotating internally. Radiography of his left shoulder showed bone erosion in the neck of the humerus. On MR imaging, a soft tissue mass was noted in the shoulder joint eroding bone. The mass showed similar signal intensity to that of surrounding muscles on T1- and T2-weighted images. At surgery, a soft, encapsulated mass was found attached to the subscapularis muscle. It was pathologically confirmed as an elastofibroma. This unusual manifestation of an elastofibroma is discussed.

Cutaneous involvement in plantar fibromatosis is very rare. The classical finding are nodules in the plantar arch, which can be detected only with palpation. A 55 year-old man presented with a 3 year history of painful plantar nodules and an ulceration. Histopathology showed a fibroblastic proliferation with a pseudosarcomatous configuration. The immunohistochemistry was positive for vimentin, alpha-actin and desmin, a pattern which characterizes a myofibroblast. Intralesional therapy with corticosteroids did not reduce the lesions. After surgical treatment, the lesions recurred.

Pseudosarcoma is a rare manifestation of Paget's disease of bone. We report the MR imaging of two cases highlighting the difficulties in diagnosis. One of the cases is the first time this condition has been described outside the long bones of the lower limb.

We present a rare case of juxtacortical chondromyxoid fibroma arising in the lesser trochanter of the right femur which corresponds to an apophysis. Radiography showed a well-defined expansive lesion with a sclerotic margin measuring 5x3.5 cm in diameter in the lesser trochanter. On spin echo T1-weighted images, the lesion revealed low signal intensity similar to muscle. On spin echo T2-weighted images, the lesion revealed high heterogeneous signal intensity, which after gadolinium injection showed heterogeneous enhancement. The inner margin of the cortex was intact and adjacent bone marrow was of normal signal intensity. The outer margin of the lesion was also clearly defined and extension into adjacent soft tissue beyond the exophytic cortical outgrowth was not evident.

A recently proposed addition to fibrous tumors in soft tissue was first described as desmoplastic fibroblastoma and later renamed collagenous fibroma. This tumor is clinically and morphologically distinct and benign. However, only a few series have been reported, and the clinicopathologic features are not widely recognized. We present two cases of collagenous fibroma of the arm. Both patients presented with an enlarging, well-circumscribed and mobile soft tissue mass. Magnetic resonance imaging showed areas of low signal intensity on both T1- and T2-weighted sequences. Needle aspiration cytology revealed nondiagnostic samples because of the low cellularity of the tumors. Each of the resected tumors was composed of low-cellular spindle- to stellate-shaped cells in a fibrous matrix with clear margination. After the marginal excisions, no recurrences were observed. Clinicians should be aware of this entity to prevent overtreatment, because imaging findings and cytologic features are similar to those of desmoid tumor.

A 60-years old female patient developed juxta-articular fibroid nodules and erythrocyanotic lesions of acrodermatitis chronica atrophicans after several tick bites. The woman was treated with ceftriaxon (Rocephin) 2 g daily parenterally without adverse reactions.

Elastofibromas are benign lesions of the chest wall. We describe the first reported case of elastofibroma in the neck. Imaging features as well as location of the lesion were atypical. On computed tomography and magnetic resonance imaging the lesion contained a marked preponderance of fat, because the lesion arose within fat.

Solitary fibrous tumors of the pleura (SFTP) are very rare neoplasms. The majority of these tumors are benign, but about 10-20 % fulfill the criteria of malignancy. The clinical presentation varies according to the size and intrathoracic localization. In early stages, often asymptomatic, the tumors may grow to an enormous size and then cause symptoms such as cough, chest pain and dyspnea, but also paraneoplastic syndromes such as hypoglycemia or digital clubbing. Between 1981 and June 1998 we treated in our institution 16 SFTP in 14 patients (4 M, 10 F, average age at first operation 58 years). Eight patients showed symptoms, whereas in the other cases the tumors were found on routine chest X-rays. The usually pedunculated SFTP were completely resected without complications. Two patients developed malignant recurrences, which infiltrated the right upper lung lobe and the diaphragm respectively. In these cases the tumor was resected together with the adjacent structures. Since late recurrences are more often malignant than primary SFTPs long-term follow-up is mandatory even in benign lesions.

We report on a 13-year-old boy who was found to have a fibroma of the tendon sheath associated with the patellar tendon and within Hoffa's fat pad of the knee. This benign tumor has never been described in this location previously. The MRI characteristics are correlated with the histologic findings.

A case of melorheostosis affecting the C7 sclerotome is described, in association with synchronous multicentric fibromatosis. The foci of fibromatosis also affected the C7 sclerotome, suggesting a similar underlying pathogenesis for both diseases.

A 3.5-year-old boy with stroke secondary to embolization of a primary cardiac tumor is presented. The diagnosis was made by two-dimensional echocardiography and confirmed intraoperatively. A papillary fibroelastoma was identified histologically. It is a rare condition and a diagnostic challenge, as patients are asymptomatic before embolization. In view of the severe consequences, prompt surgery is recommended.

Ovarian fibromatosis is very rare, and the one with focal proliferations of sex cord type elements is extraordinarily rare. A 31-year-old patient developed the bilateral ovarian enlargements and complained of lower abdominal pain, Pathology indicated that these tumors were ovarian fibromatosis with minor sex cord elements. Our two-step operation involved the first right salpingo-oophorectomy for histological examination and the subsequent enucleation of the left ovarian tumor alone. The patient conceived 10 months after operation.

Elastofibroma is a rare, benign soft-tissue tumor that occurs along the chest wall in the periscapular region. We identified five patients with a surgical diagnosis of elastofibroma who had had CT and/or MR scans. CT and MR imaging were compared to histologic findings in two patients. CT was available in three patients and demonstrated periscapular soft-tissue tumor with an attenuation similar to that of adjacent muscle. In one patient, the tumors appeared homogeneous. In two patients, the tumor showed strands of low density similar to that of adjacent subcutaneous fat on CT. On MR imaging, all five tumors showed predominantly low-signal intensity on both T1 and T2-weighted images. The tumors contained focal areas with high-signal intensity on T1-weighted scans that showed intermediate to high-signal intensity with T2-weighting. The areas of low-signal intensity on both T1 and T2-weighted images corresponded histologically to fibroelastic tissue. The areas of high signal intensity on T1-weighting and intermediate to high-signal intensity on T2-weighting reflected focal areas of fat within the tumor. Although CT and MR imaging findings are not specific, the presence of a soft-tissue tumor along the chest wall in a periscapular location that shows predominantly low intensity on all sequences reflecting fibrous tissue with interspersed focal areas of fatty tissue is highly suggestive of elastofibroma.

The clinical and histological diagnosis of sclerotic fibroma is important because of its potential association with Cowden's syndrome. Despite its distinctive histological appearance the lesion is often mis-diagnosed. We therefore present five of our own cases (2F, 3M) in which the tumor was located on the head (n = 2), arm (n = 2) and leg (n = 1), respectively. Clinically, the lesions were white to flesh-colored firm nodules ranging in size from 0.5 to 1.2 cm. None of our patients revealed any clinical evidence of Cowden's disease. Simple surgical excision seems to be curative. Histologically, they were well-circumscribed but not encapsulated dermal nodules composed of stori-form-arranged sclerosing collagen bundles and vimentin-positive fibroblastlike cells interspersed in three cases by a number of alpha-smooth-muscle actin-positive myofibroblasts. Approximately 50% of cells (dermal dendritic cells (DD)) also reacted for factor XIIIa evenly scattered throughout the lesion in contrast to the very few (< 5%) CD34+ DD found predominantly at the lower border, thus possibly reflecting the distribution of these cells in normal skin. Sclerotic fibroma expands the spectrum of fibrous lesions that may express alpha-smooth-muscle actin.

A case of an elastofibroma in the right scapular region in a 56-year-old woman was reported. The clinical and pathological findings were discussed. The location of this lesion was uncommon. The common sites were mentioned. The differential diagnosis and magnetic resonance findings were also described.

Plantar fibromatosis or Ledderhose syndrome has rarely been discussed in the medical literature. This clinical entity includes nodular Dupuytren-like indurations of the plantar aponeurosis. We present a case of a young man with isolated disease of his right foot and describe the clinical and pathomorphological features of this disease as well as its characteristic findings at magnetic resonance tomography. The therapy of choice is a subtotal resection of the plantar aponeurosis to prevent irreversible contractions of the toes. The prognosis following fascietomy is good.

Melorheostosis is a rare congenital disease, some 300 cases have been reported in the literature. The main purpose of this case report is twofold: (1) to present an unusual case of melorheostosis of the right hand, beginning in infancy, with serial radiographic studies to show the gradual progression of the disease; and (2) to present the first case with the development of "aggressive" fibromatosis (desmoid tumor) of the adjacent soft tissues, associated with melorheostosis.

Elastofibroma dorsi is a rarely made radiological diagnosis. The rarity of the condition, lack of a suitable imaging modality, and an inconsistent clinical approach in evaluating indeterminate soft tissue tumors has contributed to this failure. With magnetic resonance imaging now being recognized as the examination of choice for all indeterminate soft tissue tumors, a prebiopsy diagnosis can frequently be made by radiologists familiar with the characteristic periscapular location, predilection for elderly females, and short T2 on magnetic resonance imaging.

Since the first description of the classic triad of adenoma sebaceum, epilepsy and mental retardation by Vogt, various manifestations have been added to the clinical picture of Bourneville-Pringle disease or tuberous sclerosis. Diagnosis, therapy and potential complications of the more frequent manifestations are summarized. Since even oligosymptomatic patients may develop dangerous complications, the importance of thorough examination is emphasized.