Sample records for hemosiderotic fibrohistiocytic lipomatous

  1. Giant Hemosiderotic Dermatofibroma: A Case Report and Review of the Literature

    Marc Pusztaszeri


    Full Text Available Dermatofibroma is a common benign fibrohistiocytic lesion that usually appears as a slow-growing, firm dermal nodule with a predilection for the legs of middle-aged women. They are usually smaller than 2 cm in diameter. Many histological variants have been described. Generally, the clinical and histological diagnosis is straightforward, but differentiating it from other cutaneous tumors can be difficult in atypical cases and rare variants. Giant dermatofibroma is a rare clinical variant of dermatofibroma and less than 25 cases have been reported. It is characterized essentially by its unusually large size, which often mimics malignant tumors clinically. We first present the case of a giant hemosiderotic dermatofibroma in a 54-year-old patient, who presented with a large ulcerated mass of the left foot which was excised, and then review the literature briefly.

  2. Imaging review of lipomatous musculoskeletal lesions

    Burt Ashley M.


    Full Text Available Lipomatous lesions are common musculoskeletal lesions that can arise within the soft tissues, bone, neurovascular structures, and synovium. The majority of these lesions are benign, and many of the benign lesions can be diagnosed by radiologic evaluation. However, radiologic differences between benign and malignant lipomatous lesions may be subtle and pathologic correlation is often needed. The use of sonography, computed tomography (CT, and magnetic resonance imaging (MRI is useful not only in portraying fat within the lesion, but also for evaluating the presence and extent of soft tissue components. Lipomas make up most soft tissue lipomatous lesions, but careful evaluation must be performed to distinguish these lesions from a low-grade liposarcoma. In addition to the imaging appearance, the location of the lesion and the patient demographics can be utilized to help diagnose other soft tissue lipomatous lesions, such as elastofibroma dorsi, angiolipoma, lipoblastoma, and hibernoma. Osseous lipomatous lesions such as a parosteal lipoma and intraosseous lipoma occur less commonly as their soft tissue counterpart, but are also benign. Neurovascular and synovial lipomatous lesions are much rarer lesions but demonstrate more classic radiologic findings, particularly on MRI. A review of the clinical, radiologic, and pathologic characteristics of these lesions is presented.

  3. Hemosiderotic dermatofibroma mimicking melanoma in a 12-year-old boy: a case report.

    Acar, Emine Müge; Tad, Murat; Kilitci, Asuman; Kemeriz, Funda


    We report a case of hemosiderotic dermatofibroma presenting as a brown-black-colored nodule with peripheral extensions, which mimics melanoma. Histopathology showed completely benign features with no atypia or mitosis. Nodular extensions of childhood dermatofibromas may be related to the growth of the child not necessarily pointing to a malignant process.

  4. Concentric joint space narrowing of the hip associated with hemosiderotic synovitis (HS) including pigmented villonodular synovitis (PVNS)

    Abrahams, T.G.; Pavlov, H.; Bansal, M.; Bullough, P.


    Concentric joint space narrowing of the hip is an expected radiographic finding in cases of inflammatory arthritis such as rheumatoid arthritis or sepsis. However, similar joint space narrowing is associated with chronic hemorrhagic conditions that produce hemosiderotic synovitis. Hemosiderotic synovitis results from chronic intraarticular bleeding such as occurs in pigmented villonodular synovitis, generalized bleeding diathesis, synovial hemangioma, and chronic trauma. Five hips in five patients with concentric joint space narrowing not associated with inflammatory arthritis or with hemophilia were reviewed clinically, radiographically, and pathologically. All patients had a hemosiderotic synovitis. The definitive diagnosis of pigmented villonodular synovitis was made pathologically in two cases that demonstrated nodular areas of giant cell proliferation, collagen production, and lipid-laden histiocytes on histologic samples. (orig.)

  5. Lipomatous angiomyofibroblastoma: A 20-year literature review

    Julie Guilmette, MD


    Full Text Available Lipomatous angiomyofibroblastoma (LAMF is a rare superficial muco-cutaneous tumor of the female genital tract in women of reproductive age and early menopause. We report the case of a 36-year-old woman who presented with an asymptomatic 1 cm round vulvar mass. The tumor showed both hypercellular and hypocellular areas composed of spindle and epithelioid cells set in a loose fibromyxoid background. Anastomosing networks of small to medium sized dilated vessels, surrounded by condensed spindle cells, were distributed equally throughout the tumor. Mature adipocytes comprised more than half of the tumor's volume. The neoplastic cells were reactive for both desmin and vimentin and did not express CD34, smooth muscle actin, S100 protein and muscle-specific actin. Making the right diagnosis may be challenging to medical professionals as LAMF may mimic both clinically and histologically several other benign and malignant gynecological lesions. LAMF has a benign course without evidence of local recurrence or metastatic potential.

  6. Imaging techniques in the study of lipomatous neck lesions

    Munoz, M. M.; Herrero, C.; Garcia, M. J.; Sanchez-Lafuente, J.


    Lipomas of the neck are uncommon lesions. Those situated in deep locations and diffuse lipomatosis of the neck can be clinically mis diagnosed. We reviewed the 20 lipomatous neck lesions treated in our hospital over a 15-year period (1985-1999). Computed tomography was diagnostic in nearly 100% of cases and, this, is the method of choice for the detection and characterization of lipomatous tumors. It also provides data that aid in planning the surgical approach and enables the follow-up of clinically stable lesions that do not require surgical treatment. (Author) 11 refs

  7. Jejunojejunal Intussusception Induced by Lipomatous Polyp of Jejunum

    Jayant Kumar Biswal


    Full Text Available Adult intussusception is rare unlike childhood variety where it is the leading cause of intestinal obstruction. Preoperative diagnosis is often difficult as the symptoms are nonspecific and so high index of suspicion is needed for early diagnosis by appropriate investigations. This is a case of 65-year-old man presented with acute intestinal obstruction whose laparotomy revealed a jejunojejunal intussusception secondary to a lipomatous lesion which was successfully treated with resection and primary anastomosis. When dealing with a case of chronic intermittent intestinal obstruction, intussusception must be kept in mind as one of the differential diagnosis.

  8. Magnetic resonance of lipomatous, fibrous and muscular tissue tumors

    Dippolito, G.; Balzarini, L.; Patrillo, R.; Tess, J.T.; Musumeci, R.


    Two hundred and three MR examinations were reviewed of 195 patients with lipomatous, fibrous and muscular tissues tumors which were evaluated at staging or during the follow-up. All examinations were obtained with a 1.5 T superconductive magnet, and both T 1 and T 2 -weighted images were acquired. Its high contrast resolution, its direct multiplanarity and its allowing both T 1 and T 2 -weighted images to be obtained are the mos important characteristics of MR imaging. In our experience, MRI demonstrated a high overall accuracy (94.8%) - 94.1% at restaging alone - with similar sensitivity both at the staging of the disease (97.5%) and during the follow-up (96%). Overall sensibility was 96.3%. MR specificity in histologically proven relapses was 86.8%. Even though it is gradually assessing itself as the most important method in the evaluation of soft tissues masses, MRI allows an histological diagnosis to be made only in lipomatous tumors and in benign fibrous tumors due to their specific signal features. The commonest though specific finding is a soft tissue mass with relatively low signal intensity in T 1 -weighted images. In our opinion, MR imaging is the method of choice during the follow-up of the disease, whereas it is probably a complementary technique in the staging. (author)

  9. 110 Subfascial lipomatous tumors. MR and CT findings versus histopathological diagnosis and cytogenetic analysis

    Einarsdottir, H.; Soederlund, V.; Larsson, O.; Mandahl, N.; Bauer, H.C.F.


    Purpose: To evaluate whether liposarcoma, atypical lipomatous tumors and lipoma can be differentiated radiologically. Material and Methods: We have retrospectively analyzed CT and/or MR images of 110 subfascial lipomatous lesions. The amount of fat within the tumors was visually graded from the images as: none, 1-75%, 75-95% or 95-100%. The structure of non-fatty tumor components was compared. The images were compared to histopathology and in 37 cases to cytogenetic findings. Results: Only 4 of 20 liposarcomas contained fat. All 4 lesions, histopathologically diagnosed as atypical lipomatous tumors, contained fat but less than 75% of tumor volume. All lesions with more fat than 75% of tumor volume were histologically diagnosed as lipomas. However, one-third of the karyotyped lipomas had ring chromosomes which are considered typical for atypical lipomatous tumors. Conclusion: When a tumor is composed more or less solely of fat, the diagnosis of a lipoma or atypical lipomatous tumor with a phenotype simulating a lipoma can be assumed. When the fat content is less than 75% of the tumor volume or non-fatty nodules are found, biopsies from different tumor components have to be performed to exclude malignancy. When no fat is found, imaging does not help in differentiating lipoma or liposarcoma from other soft tissue tumors. (orig.)

  10. Right ventricular lipomatous mass and biventricular multifocal fat in a young woman: A case report

    Lee, Bo Rahm; Park, Jae Hyeong; Ahn, Kye Taek; Kim, Song Soo; Jeong, Jin Ok; Choi, Si Wan; Jin, Seon Ah; Lee, Jae Hwan


    Cardiac lipoma is a rare neoplasm of encapsulated mature adult adipose tissue. It is usually asymptomatic, but it may be related to hemodynamic obstruction depending on its location. We report a typical case of right ventricular lipomatous mass and multifocal fat infiltration of both ventricles, which were detected incidentally in a young woman.

  11. Lipomatous macrodystrophy of the ulnar nerve: Role of magnetic resonance in the diagnosis

    Capelastegui, A.; Astigarraga, E.; Galan, V.


    Lipomatous macrodystrophy is a rare cause of macrodactyly that can be included among the bening adipose tissue tumors of the peripheral nerves. We present a case involving ulnar nerve, which is an unusual location since most of the reported cases correspond to median nerve. the magnetic resonance (MR) images are analyzed, and their role in the characterization and definition of the extension of these lesions is assessed. 4 refs

  12. CT and magnetic resonance imaging finding of lipomatous hemanioperisytoma of skull base: A case report

    Park, Hee Girl; Yu, In Kyu; Kim, Han Kyu; Kim, Seung Min; Kang, Dong Wook [Eulji University Hospital, Daejeon (Korea, Republic of)


    Lipomatous hemangiopericytoma (LHPC) is recently recognized as a rare hemangiopericytoma variant. To our knowledge, imaging features of LHPC involving skull base have not yet been reported. We present the imaging features of LHPC of skull base in a 44-year-old female, along with a literature review CT and magnetic resonance imagings showed well-enhanced fatty issues containing temporal skull base masses, with pressure bony erosions.

  13. Radiological imaging features and clinicopathological correlation of hemosiderotic fibrolipomatous tumor: experience in a single tertiary cancer center

    O' Driscoll, Dearbhail; Athanasian, Edward; Hameed, Meera; Hwang, Sinchun [Memorial Sloan Kettering Cancer Center, New York, NY (United States)


    To determine the imaging features of hemosiderotic fibrolipomatous tumor (HFLT), which has a propensity towards local recurrence and the potential to transform into myxoinflammatory fibroblastic sarcoma (MIFS). The study included 8 patients with a diagnosis of HFLT and imaging at a tertiary cancer center. Imaging studies included radiographs (n = 2), ultrasound (n = 3), and MRI (n = 16). Imaging features were evaluated including location, calcification, sonographic echogenicity, vascular flow, size, border, signal characteristics, contrast enhancement, and blooming on MRI. The HFLT was located in the ankle/foot in 4 out of 8 and was subcutaneous in 8 out of 8, ranging in size from 2 to 18 cm. Histology at initial diagnosis was HFLT in 5 out of 8 and HFLT with MIFS in 3 out of 8. None was calcified on radiography. On ultrasound 2 out of 3 were heterogeneously echogenic with ≥10 foci of vascular flow. Two out of 8 patients had MRI only at local recurrence. The tumor border was infiltrative in 4 out of 6 at initial diagnosis and in 2 patients with MRI at recurrence only. Fat and septae were present in 7 out of 8 at initial diagnosis and at recurrence. Signal intensity was iso-/hypointense to muscle on T1-weighted sequences in more than two thirds of the tumor in 4 out of 7 and hyperintense to muscle in at least one third of the tumor on fluid-sensitive sequences in 6 out of 8. Contrast enhancement was heterogeneous in 7 out of 7; blooming in two thirds of the tumor on gradient-echo sequence MRI indicated hemorrhage. The HFLT commonly presents as a mass with an infiltrative border, interspersed fat and septations at initial diagnosis and local recurrence on MRI regardless of histology of HFLT alone or with MIFS. Hemosiderin deposits may be detected as blooming on gradient-echo sequences. (orig.)

  14. Angiofibroma of soft tissue with fibrohistiocytic features and intratumor genetic heterogeneity of NCOA2 gene rearrangement revealed by chromogenic in situ hybridization: a case report.

    Fukuda, Yumiko; Motoi, Toru; Kato, Ikuma; Ikegami, Masachika; Funata, Nobuaki; Ohtomo, Rie; Horiguchi, Shinichiro; Goto, Takahiro; Hishima, Tsunekazu


    Angiofibroma of soft tissue is a recently described soft tissue tumor that is characterized by fibroblastic spindle tumor cells with arborizing capillary proliferation. Cytogenetically, it harbors a specific fusion gene involving the nuclear receptor coactivator 2 (NCOA2) gene. We report here additional new pathological and cytogenetic features. A soft tissue tumor in the left thigh of 73-year-old female was investigated. Microscopically, histiocytoid tumor cells were scattered in an edematous background with branching capillary proliferation. Immunohistochemically, we identified that the tumor cells were positive for histiocytic markers such as CD68 and CD163. Rearrangement of the NCOA2 gene was detected successfully by chromogenic in situ hybridization; however, abnormal signal patterns were observed in only a small subset of tumor cells. Unlike typical tumors with bland spindle cells, the present tumor needs to be distinguished from myxoid, dendritic and clear cell tumors. This case may suggest that angiofibroma of soft tissue is not in the center of the fibroblastic/myofibroblastic tumor group, but rather shows a fibrohistiocytic nature. We also found intratumor genetic heterogeneity, which is uncommon for a translocation-associated tumor. Therefore, careful evaluation is required to detect the gene rearrangement in this tumor entity. © 2014 The Authors. Pathology International © 2014 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.

  15. Accuracy of Cytology for Diagnosis of Lipomatous Tumors: Comparison with Magnetic Resonance and Computed Tomography Findings in 175 cases

    Einarsdottir, H.; Skoog, L.; Soederlund, V.; Bauer, H.C.F.


    Purpose: To assess the value of fine-needle aspiration cytology in the diagnostic work-up of lipomatous tumors of the extremities and trunk, and to identify specific radiological features that could aid in the preoperative evaluation. Material and Methods: 175 patients with subfascial lipomatous tumors who had undergone preoperative magnetic resonance imaging or computed tomography and fine-needle aspiration cytology were studied. The percentage of fat within the lesion was visually graded from the images as: none, 1-75%, 75-95%, or 95-100%. The histological and cytological diagnoses were compared and in discordant cases the radiological images were re-reviewed. Results: There was cytological and histological concordance in 96% of lipomas and in 85% of atypical lipomatous tumors (ALT) and liposarcomas. Most discordant cases exhibited 1-75% fat. Radiological review suggested that cytological sampling problems due to tumor heterogeneity were the main cause of diagnostic difficulties. The majority of tumors with less than 75% fat were liposarcomas, and in no liposarcoma was the fat content higher than 75%. Both ALT and lipoma were found in the 95-100% group. Conclusion: Cytology can be highly accurate in the diagnosis of lipomatous tumors, including ALT; however, critical comparison with the radiological findings increases diagnostic security. In tumors with fat content visually assessed as less than 75% of the tumor volume, liposarcoma is the most likely diagnosis and a cytological diagnosis of ALT or lipoma should be questioned. In lesions with 75-95% fat, liposarcoma is unlikely, but FNAC is still indicated for safety. In lesions with 95-100% fat, FNAC is only indicated if the differentiation between lipoma and ALT influences the treatment strategy

  16. Detection of MDM2/CDK4 amplification in lipomatous soft tissue tumors from formalin-fixed, paraffin-embedded tissue: comparison of multiplex ligation-dependent probe amplification (MLPA) and fluorescence in situ hybridization (FISH).

    Creytens, David; van Gorp, Joost; Ferdinande, Liesbeth; Speel, Ernst-Jan; Libbrecht, Louis


    In this study, the detection of MDM2 and CDK4 amplification was evaluated in lipomatous soft tissue tumors using multiplex ligation-dependent probe amplification (MLPA), a PCR-based technique, in comparison with fluorescence in situ hybridization (FISH). These 2 techniques were evaluated in a series of 77 formalin-fixed, paraffin-embedded lipomatous tumors (27 benign adipose tumors, 28 atypical lipomatous tumors/well-differentiated liposarcomas, 18 dedifferentiated liposarcomas, and 4 pleomorphic liposarcomas). Using MLPA, with a cut-off ratio of >2, 36/71 samples (22 atypical lipomatous tumors/well-differentiated liposarcomas, and 14 dedifferentiated liposarcomas) showed MDM2 and CDK4 amplification. Using FISH as gold standard, MLPA showed a sensitivity of 90% (36/40) and a specificity of 100% (31/31) in detecting amplification of MDM2 and CDK4 in lipomatous soft tissue tumors. In case of high-level amplification (MDM2-CDK4/CEP12 ratio >5), concordance was 100%. Four cases of atypical lipomatous tumor/well-differentiated liposarcoma (4/26, 15%) with a low MDM2 and CDK4 amplification level (MDM2-CDK4/CEP12 ratio ranging between 2 and 2.5) detected by FISH showed no amplification by MLPA, although gain of MDM2 and CDK4 (ratios ranging between 1.6 and 1.9) was seen with MLPA. No amplification was detected in benign lipomatous tumors and pleomorphic liposarcomas. Furthermore, there was a very high concordance between the ratios obtained by FISH and MLPA. In conclusion, MLPA proves to be an appropriate and straightforward technique for screening MDM2/CDK4 amplification in lipomatous tumors, especially when a correct cut-off value and reference samples are chosen, and could be considered a good alternative to FISH to determine MDM2 and CDK4 amplification in liposarcomas. Moreover, because MLPA, as a multiplex technique, allows simultaneous detection of multiple chromosomal changes of interest, it could be in the future a very reliable and fast molecular analysis on

  17. Lipomatous muscle atrophy caused by irradiation exposure

    Rhomberg, W.; Hergan, K.


    As compared to other organs and tissues liable to sustain delayed injury from radiotherapy, the musculature seems to be a hard-wearing, radiation-resistant organ. Apart from the possibility of inducing Myodegeneratio cordis, muscles are merely threatened, as far as is known today, by possible fibrosis in the surrounding area. Certainly, extremely high doses of more than 100 Gy occasionally may trigger necrosis and atrophies in tissues. The article reports on a patient suffering from carcinoma of the bladder who developed muscle and tendon degeneration following telecobalt irradiation after a latency period of eight years, forcing him ultimately to quit work. (orig.) [de

  18. A lipomatous chest wall lesion: hibernoma

    Incedayi, M.; Sivrioglu, A.; Saglam, M.; Sonmez, G.; Tekin, L.


    Full text: A hibernoma is a rare benign soft tissue tumor derived from brown fat. The tumor is also known as 'fetal' lipoma, lipoma of embriogenic fat and lipoma of immature adipose tissue. Hibernomas are slow growing, painless soft tissue tumors which do not recur after surgical resection. Preferred locations are brown fat containing sites as thigh, inter scapular region, shoulder, axilla and mediastinum. The tumor occurs most commonly in adults, with a mean age of 38 years (age range, 2-75 years). We present a rare case of hibernoma with radiological and pathological findings

  19. Lipomatous secretory meningioma: case report and review of the literature

    Liebig, T.; Hoffmann, T.; Hosten, N.; Sander, B.; Lanksch, W.R.


    Secretory meningioma is a rare entity which may be characterised by imaging features unusual for other subtypes of meningoma, such as low attenuation on CT, high (fat-tissue equivalent) signal intensity on T1-weighted MRI, marked surrounding oedema, and irregular contrast enhancement. We report a case of secretory meningioma and review the literature. (orig.) (orig.)

  20. Unusual Thymic Hyperplasia Mimicking Lipomatous Tumor in an Eight-Year-Old Boy with Concomitant Pericardial Lipomatosis and Right Facial Hemihypertrophy

    Kim, Yoo Jin; Kim Woo Sun; Cheon, Jung Eun; Lim, Yun Jung; Kim, In One; Yeon, Kyung Mo; Jung, Kyeong Cheon; Byun, Sun Ju


    We report a case of thymic hyperplasia accompanied by pericardial lipomatosis and right facial hemihypertrophy in an 8-year-old boy. On imaging studies, the hyperplastic thymus had prominent curvilinear and nodular fatty areas simulating a fat-containing anterior mediastinal mass, which is an unusual finding in children. To our knowledge, this is the first report on a child with a combination of thymic hyperplasia, pericardial lipomatosis, and right facial hemihypertrophy. The radiologic findings are presented with a brief discussion.

  1. Computed tomography of pancreas in diabetic patients in relation to diabetic retinopathy

    Katsumata, K.; Katsumata, Y.; Sakuma, S.; Kaii, O.; Shimamoto, K.; Hirabayashi, N.; Nakagawa, T.


    Lipomatous pancreas is hardly diagnosed in living humans and usually recognized at autopsy. In the present work, it is proposed that lipomatous pancreas can be diagnosed in living humans by computed tomography (CT) of the pancreas. 2 refs.; 1 figure

  2. High-field spin-echo MR imaging of superficial and subependymal siderosis secondary to neonatal intraventricular hemorrhage

    Gomori, J.M.; Grossman, R.I.; Goldberg, H.I.; Zimmerman, R.A.; Bilaniuk, L.T.


    Two cases of superficial siderosis with subependymal siderosis, secondary to neonatal intraventricular hemorrhage, are presented. High-field spin-echo MR imaging (1.5 Tesla) showed marginal hypointensity of the ventricular walls as well as of the subpial regions. These findings were most evident on T 2 weighted images, characteristic of hemosiderotic deposits. (orig.)

  3. Liver transplantation from a deceased donor with β-thalassemia intermedia is not contraindicated: A case report.

    Gumus, Ersin; Abbasoglu, Osman; Tanyel, Cahit; Gumruk, Fatma; Ozen, Hasan; Yuce, Aysel


    The use of extended criteria donors who might have previously been deemed unsuitable is an option to increase the organ supply for transplantation. This report presents a pediatric case of a successful liver transplantation from a donor with β-thalassemia intermedia. A patient, 6-year-old female, with a diagnosis of cryptogenic liver cirrhosis underwent deceased donor liver transplantation from a thalassemic donor. Extreme hyperferritinemia was detected shortly after transplantation. The most probable cause of hyperferritinemia was iron overload secondary to transplantation of a hemosiderotic liver. Hepatocellular injury due to acute graft rejection might have contributed to elevated ferritin levels by causing release of stored iron from the hemosiderotic liver graft. Iron chelation and phlebotomy therapies were started simultaneously in the early postoperative period to avoid iron-related organ toxicity and transplant failure. Follow-up with monthly phlebotomies after discharge yielded a favorable outcome with normal transplant functions. Thalassemia intermedia patients can be candidates of liver donors to decrease pretransplant waitlist mortality. After transplantation of a hemosiderotic liver, it is important to monitor the recipient in terms of iron overload and toxicity. Early attempts to lower iron burden including chelation therapy and/or phlebotomy should be considered to avoid organ toxicity and transplant failure. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  4. Computed tomography of cystic pancreatic fibrosis

    Brachlow, M.; Zaunbauer, W.; Haertel, M.


    The computer tomographic appearances of atrophic and lipomatous degeneration of the pancreas in cystic pancreatic fibrosis are described. CT exploration of the pancreas in recommended, particularly in differential diagnostic aspects of cystic fibrosis. (orig.) [de

  5. Lipomatosis: a diverse form of hemifacial hyperplasia

    Arora, Preeti Chawla; Umarji, Hemant R.; Arora, Aman; Ramaswami, Easwaran


    A case of hemifacial hyperplasia that presented with muscular, skeletal, and dental hyperplasia along with lipomatous infiltration was described. Advanced imaging was useful in identifying the lipomatous infiltration present in the lesion, which raises the possibility of lipomatosis having a diverse presentation in hemifacial hyperplasia. As there was a scarcity of related literature in the field of dentomaxillofacial radiology, this report would make us familiar with its computed tomographic and magnetic resonance image findings.

  6. A Challenging Giant Dermatofibrosarcoma Protuberans on the Face

    Gimena Castro Pérez


    Full Text Available Dermatofibrosarcoma protuberans (DFSP is a malignant fibrohistiocytic tumor that appears exclusively on the skin. It is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence but seldom metastasizes. It may rarely occur on the head and neck accounting for less than one percent of total head and neck malignancies. We present a man with a giant DFSP on the face. Oncological, functional, and aesthetic aspects are set forth.

  7. A Benign Cardiac Growth but Not So Indolent

    Adil S. Wani


    Full Text Available Cardiac lipomatous hypertrophy is a rare benign condition that usually involves the interatrial septum. Due to its benign nature it rarely requires intervention. Its presence outside the interatrial septum is reported infrequently. We present a case of lipomatous hypertrophy in the intraventricular septum that was complicated by a severe, symptomatic, and disabling dynamic left ventricular outflow tract obstruction. The symptoms significantly improved following the excision of the mass. In our case transthoracic echocardiogram was used to visualize the mass and measure the severity of the obstruction; Cardiac Magnetic Resonance Imaging was used to characterize the mass and histopathology confirmed the diagnosis.

  8. A Benign Cardiac Growth but Not So Indolent

    Reddy, Sahadev T.; Biederman, Robert W. W.


    Cardiac lipomatous hypertrophy is a rare benign condition that usually involves the interatrial septum. Due to its benign nature it rarely requires intervention. Its presence outside the interatrial septum is reported infrequently. We present a case of lipomatous hypertrophy in the intraventricular septum that was complicated by a severe, symptomatic, and disabling dynamic left ventricular outflow tract obstruction. The symptoms significantly improved following the excision of the mass. In our case transthoracic echocardiogram was used to visualize the mass and measure the severity of the obstruction; Cardiac Magnetic Resonance Imaging was used to characterize the mass and histopathology confirmed the diagnosis. PMID:27293911

  9. Gastric cancer metastatic to neck lipoma. A case report with imaging consideration

    Nomura, Shuko [Tohoku University Hospital, Department of Radiology, Sendai, Miyagi (Japan); National Sendai Medical Center, Department of Radiology, Sendai, Miyagi (Japan); Kurihara, Noriko [National Sendai Medical Center, Department of Radiology, Sendai, Miyagi (Japan); Ishikawa, Tomohiko; Tateda, Masaru [National Sendai Medical Center, Department of Otorhinolaryngology, Sendai, Miyagi (Japan); Sakurada, Junko [National Sendai Medical Center, Department of Pathology, Sendai, Miyagi (Japan)


    A 71-year-old man visited our hospital for examination of a soft neck mass. Computed tomography and magnetic resonance imaging scans showed a well-circumscribed large lipomatous tumor with multiple nodules inside. Atypical lipomatous tumor or lipoma involving the lymph nodes was considered. Pathological examination of the surgical specimen suggested typical lipoma including multiple metastatic foci from gastric cancer. Subsequent endoscopy revealed a gastric tumor, which was histologically proven to be signet-ring cell carcinoma. Considering these findings, this case was diagnosed as tumor-to-tumor metastasis to lipoma. We present the first such case with imaging and clinical characteristics. (orig.)

  10. Simultaneous Epstein Barr Virus and Cytomegalovirus Infection Accompanied by Leiomyomatous Change in a Well-Differentiated Liposarcoma in a Patient With Long-Term Corticosteroid Treatment

    Harald J. Hoekstra


    Full Text Available Patient. A 59-year-old woman presented with a large tumour of the abdominal wall. She had been taking corticosteroids for severe chronic obstructive pulmonary disease for 15 years. On CT scan the tumour had the characteristics of lipomatous tissue with a dense core.

  11. Simultaneous epstein barr virus and cytomegalovirus infection accompanied by leiomyomatous change in a well-differentiated liposarcoma in a patient with long-term corticosteroid treatment.

    Sars, P R; Molenaar, W M; Koudstaal, J; Hoekstra, H J


    Patient. A 59-year-old woman presented with a large tumour of the abdominal wall. She had been taking corticosteroids for severe chronic obstructive pulmonary disease for 15 years. On CT scan the tumour had the characteristics of lipomatous tissue with a dense core.Results. Histology showed a

  12. MR imaging of lipoma and liposarcoma

    Einarsdottir, H.; Soederlund, V.; Larson, O.; Jenner, G.; Bauer, H.C.F.


    Purpose: To evaluate whether lipoma, atypical lipomatous tumors, and liposarcomas can be differentiated by MR images. Material and Methods: The MR images of 59 lipomatous lesions and liposarcomas were retrospectively reviewed. Apart from size, surgical site, location and margins, the percentage of fat of the tumor volume was assessed as none, 1-75%, 75-95%, or 95-100%. Results: None of the 18 liposarcomas contained fat that could be recognized by MR imaging. The 3 atypical lipomatous tumors all contained fat but less than 75% of the tumor volume. In 32 of 38 ordinary lipomas, the percentage of fat was 95-100%, and in 4 less than 95% of the tumor volume. Two lipomas did not contain fat that could be recognized by MR imaging. Conclusion: A lesion which predominantly has a fat signal is, in all probability, an ordinary lipoma. Lesions with less fat, but still mostly fatty, may either be lipoma or atypical lipomatous tumor. In this group, the discrimination between these two entities cannot be based upon imaging features. In the absence of a fat signal, liposarcoma or lipoma cannot be differentiated from other soft tissue tumors. (orig.)

  13. Long-Term Follow-up of Recurrence and Patient Satisfaction After Surgical Treatment of Gynecomastia.

    Fricke, A; Lehner, G M; Stark, G B; Penna, V


    "Gynecomastia" is an enlargement of the male breast. Our study aims to assess patient satisfaction as well as evaluate differences in recurrence rates in lipomatous and glandular gynecomastia 10-19 years postoperatively. Forty-one gynecomastia patients undergoing surgical treatment from 1997 to 2005 were invited for a follow-up examination 10-19 years postoperatively. Of these, 16 patients presented for a clinical examination. Patient satisfaction was measured with a validated questionnaire [consultation satisfaction questionnaire (CSQ)-9]. Furthermore, photo-material and patient charts were evaluated concerning preoperative macroscopical type of gynecomastia, BMI, and operative technique. Mean follow-up time was 13.8 years (range: 10.5-19 years). Eight patients (50%) had presented with lipomatous and eight patients (50%) with glandular gynecomastia prior to surgery. One of the patients with glandular gynecomastia (12.5%) presented with recurrence at the time of follow-up, while five of the eight patients showing lipomatous gynecomastia (62.5%) presented with recurrence. Interestingly, younger patient groups tend to be more satisfied with the operative treatment of gynecomastia than older patient groups, especially regarding the improvement of self-esteem. Long-term follow-up results showed that recurrence rates are significantly higher in patients with lipomatous gynecomastia than in patients with glandular gynecomastia, with BMI increase in patients with glandular and lipomatous gynecomastia showing no statistically significant differences. Furthermore, general patient satisfaction and improvement of self-esteem was higher in younger patient groups than older patient groups. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors . DRKS00009630.

  14. Characterising benign fibrous soft-tissue tumours in adults: why is it so difficult and what do we need to know?

    Ng, E.; Tandon, A.A.; Ho, B.C.S.; Chong, B.K.


    Fibrous, myofibroblastic, and fibrohistiocytic soft-tissue tumours are amongst the most common benign soft-tissue lesions encountered in clinical practice. They demonstrate varied biological behaviour and imaging characteristics. Benign fibroblastic lesions, such as nodular fasciitis, are small, have a self-limited course, and rarely recur after excision, whereas deep fibromatosis and plexiform fibrohistiocytic tumours tend to exhibit more aggressive features and often have high recurrence rates. MRI with its superior tissue contrast, multiplanar imaging capability, and lack of ionising radiation is regarded as the preferred method of tumour evaluation, tissue characterisation, and assessment of treatment response. Histopathological features are depicted at MRI, reflecting the amount and distribution of the cellular and fibrous matrix. Cellular tumours tend to show higher T2 signal intensity and post-contrast enhancement as compared to tumours with greater collagenous content, which appear dark and show less enhancement. Awareness of MR characteristics, pathological behaviour, and common sites of occurrence of fibrous soft-tissue tumours will help radiologists to determine the appropriate differential diagnosis and guide patient management

  15. Hepatic capillariasis in two captive Callithrix penicillata

    Washington Luiz Assunção Pereira


    Full Text Available This study aimed to describe the occurrence of hepatic capillariasis in two non-human primates of the species Callithrix penicillata that were kept in captivity. Case 1 was in a young female, whose liver presented pale coloring, softening due to autolysis and several noticeably whitish areas. Case 2 was in a male of unknown age, whose liver was slightly pale and brownish. Histological analysis on the samples revealed extensive interstitial fibrosis with hemosiderotic areas. Adult nematodes of Capillaria hepatica and numerous eggs, morphologically characterized by their non-embryonic ellipsoid shape, with bipolar plugs that did not protrude from the shell, thick and birefringent, with a porous layer, thus giving a striated appearance to the eggs. Some cystic formations presented nematode eggs surrounded by numerous foreign-body giant cells. The literature consulted made little reference to reports of Capillaria hepatica in non-human primates. However, similar infections have been extensively studied in domestic rodents.

  16. Hepatic lipomas and steatosis: An association beyond chance

    Martin-Benitez, Gregorio; Marti-Bonmati, Luis; Barber, Carmen; Vila, Rocio


    Objective: To determine if hepatic lipomas have a higher prevalence of liver steatosis than other benign hepatic lesions. Materials and methods: Ninety-two benign hepatic lesions were analyzed with magnetic resonance (MR) imaging. There were 6 lipomas and 86 benign non-lipomatous lesions, including 55 hemangiomas, 23 focal nodular hyperplasias (FNH) and 8 adenomas. All studies included a chemical shift T1-weighted sequence (in-phase and opposed-phase) in order to evaluate the presence of steatosis. Results: A statistically significant relationship (Fischer's Exact Test, p = 0.019) between hepatic lipomas and steatosis was demonstrated. Fifty percent of hepatic lipomas associated steatosis, while this association was present in only 9% of the non-lipomatous lesions. Conclusion: Lipomas have a significantly greater association with steatosis when compared to nonlipomatous lesions. This relationship may be related to a common insuline resistance mechanism.

  17. Dedifferentiated liposarcoma of the anterior mediastinum. A rare case

    Harth, S.; Litzlbauer, H.D.; Behrens, C.B.; Roller, F.C.; Gamerdinger, U.; Burchert, D.; Krombach, G.A.


    Liposarcoma accounts for approximately 14 % of all malignant soft-tissue tumors, regardless of anatomical location (Kransdorf MJ et al. Imaging of soft tissue tumors. Philadelphia: Lippincott Williams and Wilkins, 2014). Primary mediastinal liposarcomas are rare. Liposarcoma is classified into four histologic subtypes: Myxoid/round cell, pleomorphic, atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma. Dedifferentiated liposarcoma occurs most commonly in the retroperitoneum and in the soft tissues of the extremities. Like atypical lipomatous tumor/well-differentiated liposarcoma, it is characterized by amplification of MDM2 and CDK4 genes on chromosome 12 (Crago AM et al. Curr Opin Oncol 2011; 23: 373 - 378). Possible symptoms of mediastinal liposarcoma are dyspnea, wheezing, chest pain, cough, superior vena cava syndrome, and weight loss (Macchiarini P et al. Lancet Oncol 2004; 5: 107 - 118).

  18. Dedifferentiated liposarcoma of the anterior mediastinum. A rare case

    Harth, S.; Litzlbauer, H.D.; Behrens, C.B.; Roller, F.C.; Gamerdinger, U.; Burchert, D.; Krombach, G.A.


    Liposarcoma accounts for approximately 14 % of all malignant soft-tissue tumors, regardless of anatomical location (Kransdorf MJ et al. Imaging of soft tissue tumors. Philadelphia: Lippincott Williams and Wilkins, 2014). Primary mediastinal liposarcomas are rare. Liposarcoma is classified into four histologic subtypes: Myxoid/round cell, pleomorphic, atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma. Dedifferentiated liposarcoma occurs most commonly in the retroperitoneum and in the soft tissues of the extremities. Like atypical lipomatous tumor/well-differentiated liposarcoma, it is characterized by amplification of MDM2 and CDK4 genes on chromosome 12 (Crago AM et al. Curr Opin Oncol 2011; 23: 373 - 378). Possible symptoms of mediastinal liposarcoma are dyspnea, wheezing, chest pain, cough, superior vena cava syndrome, and weight loss (Macchiarini P et al. Lancet Oncol 2004; 5: 107 - 118).

  19. Giant intramuscular lipoma of the tongue: a case report and literature review

    Colella, Giuseppe; Biondi, Paolo; Caltabiano, Rosario; Vecchio, Giada Maria; Amico, Paolo; Magro, Gaetano


    We herein report a rare case of giant intramuscular lipoma of the tongue. A 75-year-old Italian male presented at our department with a large tumor at the tip of the tongue that had been present for over 30 years. Clinical examination revealed a yellowish lesion, measuring 10 cm in maximum diameter, protruding from lingual surface. Histological examination showed an unencapsulated lipomatous tumor composed of mature adipocytes, uniform in size and shape, diffusely infiltrating striated muscle...

  20. Idiopathic epidural lipomatosis as a cause of pain and neurological symptoms attributed initially to radiation damage.

    Millwater, C J; Jacobson, I; Howard, G C


    Epidural lipomatosis is a rare condition in which overgrowth of extradural fat can lead to back pain, spinal cord compression and radiculopathy. A 51-year-old man developed back pain and reduced mobility following a standard course of radiotherapy for a Stage I seminoma. His symptoms and radiological appearances were initially attributed to radiation fibrosis. Further investigations and operative intervention revealed epidural lipomatosis. The excess lipomatous tissue was removed with complete resolution of his symptoms.

  1. CT and MR imaging of high cervical intradural lipomas

    Oh, Joo Hyeong; Choi, Woo Suk; Lee, Sun Wha; Lim, Jae Hoon; Leem, Woon; Kim, Gook Ki; Rhee, Bong Arm [Kyung Hee University Hospital, Seoul (Korea, Republic of)


    Intradural spinal lipoma occurs in less than 1% of all spinal cord tumors. It has been described at every level of the spinal canal, although its most common location is the cervicothoracic and thoracic region. However, lipoma located in the high cervical region is very unusual. We described two cases, a teenager and an adult, with progressive neurologic deficit from such a lipomatous tumor, which were evaluated by CT scanning and MR imaging.

  2. Magnetic resonance imaging of lipoma and liposarcoma: potential of short tau inversion recovery as a technique of fat suppression

    Pang, A.K.K.; Hughes, T.


    The present limited retrospective study was performed to assess MR imaging of lipomatous tumours of the musculoskeletal system and to evaluate the potential of the T2 short tau inversion-recovery (STIR) technique for differentiating lipomas from liposarcomas. Magnetic resonance imaging of 12 patients with lipomatous tumours of the musculoskeletal system (eight benign lipomas, three well differentiated liposarcomas and one myxoid liposarcoma) were reviewed. Benign lipomas were usually superficial and showed homogeneity on T1- and T2-weighted spin echo sequences. Full suppression at T2-STIR was readily demonstrated. In contrast the liposarcomas in the present series were all deep-seated. Two well-differentiated liposarcomas showed homogeneity at long and short relaxation time (TR) but failed to show complete suppression at T2-STIR. One case of well-differentiated liposarcoma (dedifferentiated liposarcoma) and one of myxoid liposarcoma showed mild and moderate heterogeneity at T1 and T2, respectively and posed no difficulty in being diagnosed correctly. In conclusion, short and long TR in combination with T2 STIR show promise in differentiating benign from malignant lipomatous tumours of the musculoskeletal system, when taken in combination with the position of the tumour. Copyright (1999) Blackwell Science Pty Ltd

  3. Localized Pigmented Villonodular Synovitis of the Hip: Sudden-Onset Pain Caused by Torsion of the Tumor Pedicle

    Kiyokazu Fukui


    Full Text Available Pigmented villonodular synovitis is a rare, benign, but potentially locally aggressive disease that should be considered in younger patients who present with monoarticular joint symptoms and pathology. We present the case of a 33-year-old woman with a mass arising from her right hip joint that was examined using a multimodal radiological approach. Because her clinical presentation mimicked that of synovial osteochondromatosis of the hip, surgical dislocation was performed. Histopathological examination of the resected specimen confirmed the diagnosis of localized pigmented villonodular synovitis, with the mass consisting of proliferation of fibrohistiocytic cells, abundant hemosiderin, foamy histiocytes, and occasional giant cells. Because of the presence of tumor necrosis, we hypothesize that torsion of the tumor pedicle was the cause of acute presentation.

  4. Mast cells in cutaneous tumors: innocent bystander or maestro conductor?

    Biswas, Asok; Richards, Joanna E; Massaro, Joseph; Mahalingam, Meera


    Evidence favoring a critical role for mast cells (MC) in cutaneous malignancies is conflicting. Using the immunohistochemical stain tryptase, MC counts were performed in the following tumor categories: epithelial (basal cell carcinoma [BCC]: nodular [N], n = 10, infiltrative [I], n = 10; squamous cell carcinoma [SCC]: well differentiated [W], n = 9, moderate/poorly differentiated [MP], n = 15); melanocytic (intradermal nevus, n = 10, malignant melanoma in situ [MMIS], n = 8, invasive melanoma, n = 15); vascular (hemangioma [HEM], n = 11, Kaposi's sarcoma [KS], n = 14, angiosarcoma [AS] n = 8); and fibrohistiocytic (dermatofibroma [DF], n = 7, atypical fibroxanthoma [AFX], n = 5, dermatofibrosarcoma protuberans [DFSP], n = 5). MC (intra- and peritumoral) were expressed as cells per 10 high-power fields. Mean MC counts were: BCCN 166.30; BCCI 130; SCCW 167.22; SCCMP 133.80; nevus 156.40; MMIS 93; MM radial growth phase 73.86; MM vertical growth phase 82.13; HEM 165.18; KS 120.57; AS 168.13; DF 247.86; AFX 280.20; and DFSP 83.60. Using a one-way analysis of variance, statistically significant differences were observed in the following pairs: AFX and DF vs. DFSP, nevus vs. melanoma, AS and HEM vs. Our findings appear to point towards a dichotomous role for mast cells in fibrohistiocytic and vascular neoplasms and argue against their preferential recruitment in epithelial malignancies and malignant melanoma. The value of mast cell counts as a prognostic index appears to be limited in most cutaneous malignancies. © 2013 The International Society of Dermatology.

  5. Giant intramuscular lipoma of the tongue: a case report and literature review.

    Colella, Giuseppe; Biondi, Paolo; Caltabiano, Rosario; Vecchio, Giada Maria; Amico, Paolo; Magro, Gaetano


    We herein report a rare case of giant intramuscular lipoma of the tongue. A 75-year-old Italian male presented at our department with a large tumor at the tip of the tongue that had been present for over 30 years. Clinical examination revealed a yellowish lesion, measuring 10 cm in maximum diameter, protruding from lingual surface. Histological examination showed an unencapsulated lipomatous tumor composed of mature adipocytes, uniform in size and shape, diffusely infiltrating striated muscle fibers of the tongue. The patient is well with no local recurrence after a 15-month follow-up period.

  6. Intraosseous Hibernoma in the Sacrum of an Adult.

    Westacott, Lorraine; Collins, Angus; Dickenson, Ian


    Hibernoma is an uncommon lipomatous tumor composed of brown adipose cells that until recently has only ever been described in soft tissue locations. This report describes the histological features of a discrete lesion in the sacrum of an 84-year-old lady. Biopsy of this lesion demonstrated that it consisted predominantly of multivacuolated lipocytes with a morphological and immunohistochemical profile consistent with brown fat cells. The lesion was diagnosed as an intraosseous hibernoma. This is the 16th report in the English literature of a hibernoma occurring outside of a soft tissue location. © The Author(s) 2016.

  7. A case of fibrolipomatous hamartoma of the median nerve with macrodactyly

    Sathish Arakeri


    Full Text Available Fibrolipomatous hamartoma of nerve is a tumor-like lipomatous process principally involving affecting young persons. The median nerve is most commonly affectedinvolved. The lesion is characterized by a soft slowly growing mass, surrounding and infiltrating major nerves and their branches. It may cause symptoms of compression neuropathy and is associated with macrodactyly in one third of cases. Here, we present a case of Fibrolipomatous hamartoma of nerve in the wrist of a young man arising from median nerve. Debulking of the tumour was performed.

  8. Radiological diagnostics of muscle diseases

    Weber, M.A.; Essig, M.; Kauczor, H.U.


    Muscular diseases are a heterogeneous group of diseases with difficult differential diagnosis. This article reviews morphological and functional radiological techniques for assessment of muscular diseases. Morphological techniques can describe edema-like changes, lipomatous and atrophic changes of muscular tissue. However, these imaging signs are often not disease-specific. As a result, clinicians assign radiology a secondary role in the management of muscular diseases. Meanwhile, functional radiological techniques allow the assessment of muscle fiber architecture, skeletal muscle perfusion, myocellular sodium-homoeostasis, lipid- and energy-phosphate metabolism, etc. By detecting and spatially localizing pathophysiological phenomena, these new techniques can increase the role of radiology in muscular diseases. (orig.)

  9. The value of fat-suppressed T2 or STIR sequences in distinguishing lipoma from well-differentiated liposarcoma

    Galant, J. [Servicio de Radiodiagnostico, Hospital Universitario San Juan de Alicante, Ctra. Nacional 332 Alicante-Valencia s/n, 03550 San Juan de Alicante (Spain); Resonancia Magnetica del Sureste, Murcia (Spain); Marti-Bonmati, L. [Department of Radiology, Hospital Universitario Dr. Peset, Valencia (Spain); Saez, F. [Department of Radiology, Hospital Cruces de Baracaldo, Vizcaya (Spain); Soler, R. [Department of Radiology, Hospital Juan Canalejo, A Coruna (Spain); Alcala-Santaella, R. [Department of Traumatology, Hospital Universitario San Juan de Alicante, Ctra. Nacional 332 Alicante-Valencia s/n, 03550 San Juan de Alicante (Spain); Navarro, M. [Servicio de Radiodiagnostico, Hospital Universitario San Juan de Alicante, Ctra. Nacional 332 Alicante-Valencia s/n, 03550 San Juan de Alicante (Spain)


    The objective of this study was to evaluate the diagnostic value of fat-suppressed T2-weighted (FS-T2) images or short tau inversion recovery (STIR) imaging in distinguishing lipoma from lipoma-like subtype of well-differentiated liposarcoma. Spin-echo T1-weighted and STIR or fat-suppression T2-weighted sequences were performed in 60 lipomas and 32 lipoma-like well-differentiated liposarcomas, histologically proven, looking for thick septa or nodules in T1-weighted images and linear, nodular, or amorphous hyperintensities on FS-T2/STIR sequences. Fourteen lipomas (23.3%) showed thick septa and/or nodules on T1, whereas on FS-T2 or STIR sequences only seven (11.7%) displayed hyperintense nodules and/or septa. All well-differentiated liposarcomas contained these signs on FS-T2 or STIR sequences. The presence of hyperintense septa or nodules in a predominantly lipomatous tumor on FS-T2/STIR sequences helps to differentiate malignant tumors from lipomas. Employing the presence of hyperintense nodules and/or septa as criteria of malignancy specificity was 76.6% and sensitivity 100%. Overdiagnoses of well-differentiated liposarcoma can occur due to the presence of non-lipomatous areas within lipomas. (orig.)

  10. CLOVES syndrome: review of a PIK3CA-related overgrowth spectrum (PROS).

    Martinez-Lopez, A; Blasco-Morente, G; Perez-Lopez, I; Herrera-Garcia, J D; Luque-Valenzuela, M; Sanchez-Cano, D; Lopez-Gutierrez, J C; Ruiz-Villaverde, R; Tercedor-Sanchez, J


    Overgrowth syndromes are characterized by global or localized disproportionate growth associated with other anomalies, including vascular malformations and neurological and/or visceral disorders. CLOVES (Congenital Lipomatous asymmetric Overgrowth of the trunk with lymphatic, capillary, venous, and combined-type Vascular malformations, Epidermal naevi, Scoliosis/Skeletal and spinal anomalies) is an overgrowth syndrome caused by mosaic activating mutation in gene PIK3CA, which gives rise to abnormal PI3K-AKT-mTOR pathway activation. These mutations are responsible for the clinical manifestations of the syndrome, which include low- and high-flow vascular malformations, thoracic lipomatous hyperplasia, asymmetric growth, and visceral and neurological disorders. These common anomalies are illustrated with figures from two personal cases. Identification of the clinical and genetic characteristics of CLOVES syndrome is crucial for the differential diagnosis with other overgrowth syndromes, such as Proteus or Klippel-Trenaunay (K-T) syndromes, and for the therapeutic management of the different anomalies. In this context, a new entity comprising different syndromes with phenotypic mutations in PIK3CA has been proposed, designated PIK3CA-related overgrowth spectrum (PROS), with the aim of facilitating clinical management and establishing appropriate genetic study criteria. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  11. The value of fat-suppressed T2 or STIR sequences in distinguishing lipoma from well-differentiated liposarcoma

    Galant, J.; Marti-Bonmati, L.; Saez, F.; Soler, R.; Alcala-Santaella, R.; Navarro, M.


    The objective of this study was to evaluate the diagnostic value of fat-suppressed T2-weighted (FS-T2) images or short tau inversion recovery (STIR) imaging in distinguishing lipoma from lipoma-like subtype of well-differentiated liposarcoma. Spin-echo T1-weighted and STIR or fat-suppression T2-weighted sequences were performed in 60 lipomas and 32 lipoma-like well-differentiated liposarcomas, histologically proven, looking for thick septa or nodules in T1-weighted images and linear, nodular, or amorphous hyperintensities on FS-T2/STIR sequences. Fourteen lipomas (23.3%) showed thick septa and/or nodules on T1, whereas on FS-T2 or STIR sequences only seven (11.7%) displayed hyperintense nodules and/or septa. All well-differentiated liposarcomas contained these signs on FS-T2 or STIR sequences. The presence of hyperintense septa or nodules in a predominantly lipomatous tumor on FS-T2/STIR sequences helps to differentiate malignant tumors from lipomas. Employing the presence of hyperintense nodules and/or septa as criteria of malignancy specificity was 76.6% and sensitivity 100%. Overdiagnoses of well-differentiated liposarcoma can occur due to the presence of non-lipomatous areas within lipomas. (orig.)

  12. Septum-like structures in lipoma and liposarcoma: MR imaging and pathologic correlation

    Hosono, M.; Kobayashi, H.; Fujimoto, R.; Kotoura, Y.; Tsuboyama, T.; Matsusue, Y.; Nakamura, T.; Itoh, T.; Konishi, J.


    Objective. To investigate the septum-like structures in predominantly lipomatous tumors, by correlating fat-suppressed MR images with histopathologic findings. Design and patients. The MR findings of three cases of well-differentiated liposarcoma (atypical lipoma), one case of lipoma-like component of dedifferentiated liposarcoma, and nine cases of lipoma were analyzed. T1-, T2-, and fat-suppressed T1-weighted images after Gd-DTPA administration were obtained. Surgical specimens from five patients (four with liposarcoma and one with lipoma) were also scanned with a MR unit, and compared with the pathologic findings. Results and conclusions. Enhancement features of lipoma and liposarcoma were well visualized on fat-suppressed T1-weighted images after Gd-DTPA administration. The septum-like structures of liposarcoma are thick and enhanced considerably, while septa of lipoma are thin and enhanced only slightly. Pathologically, the septum-like structures of liposarcoma contained muscle fibers and the septa of lipoma represented fibrous capsule. Identification of well-enhanced septa in a predominantly lipomatous tumor helps to differentiate malignant tumors from lipomas. As the septum-like structures of liposarcoma contain a skeletal muscle component the tumor might need more extensive surgical procedures including resection of adjacent muscles. (orig.). With 4 figs., 1 tab

  13. Isolated pulmonary involvement in Erdheim–Chester disease

    Enambir Singh Josan


    Full Text Available Erdheim–Chester disease is a rare non-Langerhans cell histiocytic disorder. It is primarily a disease of the long bones. Pulmonary involvement in systemic disease is detected in about half the reported cases. Isolated lung involvement is extremely rare with no clear recommendations for treatment. A 52-year-old caucasian male was evaluated for 1.9 cm × 1.6 cm spiculated nodule in the right upper lobe. Pulmonary function testing and bronchoscopy with endobronchial ultrasound, transbronchial biopsy, and microbiology were inconclusive. Positron emission tomography–computed tomography (PET-CT was significant for the avidity in same lung nodule along with mediastinal and hilar adenopathy but no bone involvement. Wedge resection with histopathology and immunohistochemistry reported a fibrohistiocytic infiltrate in bronchovascular distribution which was positive for CD68 and negative for CD1A, S100, and BRAF V600E mutation. Magnetic resonance imaging brain ruled out central nervous system involvement. The rarity of the condition along with the complex pathology makes it difficult to diagnose and hence intervene appropriately.

  14. Splenic masses in dogs. Part 1: Epidemiologic, clinical characteristics as well as histopathologic diagnosis in 249 cases (2000-2011).

    Eberle, N; von Babo, V; Nolte, I; Baumgärtner, W; Betz, D


    Splenic masses have a high prevalence and are more common than diffuse splenic enlargement in dogs. It was the aim of the present study to retrospectively describe clinical aspects and histopathologic characteristics of dogs with splenic masses. Records of patients with a histologically diagnosed splenic mass between January 2000 and March 2011 were reviewed. 249 dogs met the inclusion criteria and could be included in the study. Splenic masses were diagnosed histologically as non-malignant disease (n=117; 47%) and malignant splenic disease (n=132; 53%). Hemangiosarcoma was the most common histological diagnosis (n=97; 73.5%). Other malignant tumors included sarcoma (n=14), fibrohistiocytic nodules (n=9) as well as lymphoma, blastoma and adenocarcinoma. The non-malignant masses consisted of nodular hyperplasia (n=60), splenic hematoma (n=41), and splenitis (n=6). Dogs with hemoperitoneum had a higher frequency of splenic neoplasia. The results corroborate previous findings that hemangiosarcoma is the most frequent neoplasm of the canine spleen. However, in approximately half of the cases benign lesions were histologically diagnosed. It is essential that a frank discussion is held with owners regarding the prognosis associated with the treatment of dogs with a splenic mass associated with hemoperitoneum.

  15. Molecular prevalence of Bartonella, Babesia, and hemotropic Mycoplasma sp. in dogs with splenic disease.

    Varanat, M; Maggi, R G; Linder, K E; Breitschwerdt, E B


    Among diseases that cause splenomegaly in dogs, lymphoid nodular hyperplasia (LNH), splenic hemangiosarcoma (HSA), and fibrohistiocytic nodules (FHN) are common diagnoses. The spleen plays an important role in the immunologic control or elimination of vector-transmitted, blood-borne pathogens, including Bartonella sp., Babesia sp., and hemotropic Mycoplasma sp. To compare the prevalence of Bartonella sp., Babesia sp., and hemotropic Mycoplasma sp. DNA in spleens from dogs with LNH, HSA, and FHN. Paraffin-embedded, surgically obtained biopsy tissues from LNH (N = 50), HSA (N = 50), and FHN (N = 37) were collected from the anatomic pathology archives. Spleens from specific pathogen-free (SPF) dogs (N = 8) were used as controls. Bartonella sp., Babesia sp., and Mycoplasma sp. DNA was amplified by PCR, followed by DNA sequencing. Bartonella sp. DNA was more prevalent in FHN (29.7%) and HSA (26%) as compared to LNH (10%) (P = .019, .0373, respectively) or control spleens (0.0%). The prevalence of Babesia sp. and hemotropic Mycoplasma sp. DNA was significantly lower than Bartonella sp. DNA in HSA (P = .0005, .006, respectively) and FHN (P = .003, .0004, respectively). There was no statistically significant difference in DNA prevalence among the 3 genera in the LNH group. The higher prevalence of Bartonella sp. in FHN and HSA warrants future investigations to determine if this bacterium plays a role in the development of these splenic diseases. Copyright © 2011 by the American College of Veterinary Internal Medicine.

  16. Fibroblastic connective tissue nevus: Clinicopathological and immunohistochemical study of 14 cases.

    Pennacchia, Ilaria; Kutzner, Heinz; Kazakov, Dmitry V; Mentzel, Thomas


    We present herein a series of 14 lesions showing overlapping features with the newly defined benign cutaneous mesenchymal neoplasm labeled as fibroblastic connective tissue nevus (FCTN). Total of 8 patients were male and 5 were female, ranging in age from 1 to 56 years. Lesions appeared as isolated nodules or plaques on the trunk (7 cases), the limbs (4 cases) and the neck (2 cases). Histologically, all cases were composed of bundles of bland spindle cells of fibroblastic/myofibroblastic lineage irregularly branching within the reticular dermis and along fibrous septa in the subcutis. Adnexal structures and dermal adipocytes were entrapped by the fascicles, the epidermis was often papillomatous and elastic fibers were decreased and fragmented. Expression of CD34 and ASMA was found in 8 and 7 cases, respectively. Follow-up was available for 7 patients (mean follow-up, 5 years; range, 1-10 years). None of the cases metastasized or recurred, even when incompletely excised. The differential diagnosis of FCTN is broad and includes hypertrophic scar, dermatofibroma, dermatomyofibroma, pilar leiomyoma, plaque-stage DFSP, CD34-positive plaque-like dermal fibroma, fibroblastic-predominant plexiform fibrohistiocytic tumor, lipofibromatosis, superficial desmoid fibromatosis and fibrous hamartoma of infancy, of which it represents probably the monophasic variant. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  17. Serum ferritin, serum nitric oxide, and cognitive function in pediatric thalassemia major

    Septiana Nur Qurbani


    Full Text Available Background Hemolysis and repeated blood transfusions in children with thalassemia major cause iron overload in various organs, including the brain, and may lead to neurodegeneration. Hemolysis also causes decreased levels of nitric oxide, which serves as a volume transmitter and slow dynamic modulation, leading to cognitive impairment. Objective To assess for correlations between serum ferritin as well as nitric oxide levels and cognitive function in children with thalassemia major.  Methods This analytical study with cross-sectional design on 40 hemosiderotic thalassemia major patients aged 6−14 years, was done at the Thalassemia Clinic in Dr. Hasan Sadikin Hospital, Bandung, West Java, from May to June 2015. Serum ferritin measurements were performed by an electrochemiluminescence immunoassay; serum nitric oxide was assayed by a colorimetric procedure based on Griess reaction; and cognitive function was assessed by the Wechsler Intelligence Scale for Children test. Statistical analysis was done using Spearman’s Rank correlation, with a significance value of 0.05. Results Abnormal values in verbal, performance, and full scale IQ were found in 35%, 57.5% and 57.5%, respectively. Serum nitric oxide level was significantly correlated with performance IQ (P=0.022, but not with verbal IQ (P=0.359 or full scale IQ (P=0.164. There were also no significant correlations between serum ferritin level and full scale, verbal, or performance IQ (P=0.377, 0.460, and 0.822, respectively. Conclusion Lower serum nitric oxide level is significantly correlated to lower cognitive function, specifically in the performance IQ category. However, serum ferritin level has no clear correlation with cognitive function.

  18. LABILE IRON IN CELLS AND BODY FLUIDS . Physiology, Pathology and Pharmacology

    Zvi Ioav Cabantchik


    Full Text Available In living systems iron appears predominantly associated with proteins, but can also be detected in forms referred as labile iron, which denotes the combined redox properties of iron and its amenability to exchange between ligands, including chelators. The labile cell iron (LCI composition varies with metal concentration and substances with chelating groups but also with pH and the redox potential. Although physiologically in the lower µM range, LCI plays a key role in cell iron economy as cross-roads of metabolic pathways. LCI levels are continually regulated by an iron-responsive machinery that balances iron uptake versus deposition into ferritin. However, LCI rises aberrantly in some cell types due to faulty cell utilization pathways or infiltration by pathological iron forms that are found in hemosiderotic plasma. As LCI attains pathological levels, it can catalyze reactive O species (ROS formation that, at particular threshold, can surpass cellular anti-oxidant capacities and seriously damage its constituents. While in normal plasma and interstitial fluids, virtually all iron is securely carried by circulating transferrin (that renders iron essentially non-labile, in systemic iron overload (IO, the total plasma iron binding capacity is often surpassed by a massive iron influx from hyperabsorptive gut or from erythrocyte overburdened spleen and/or liver. As plasma transferrin approaches iron saturation, labile plasma iron (LPI emerges in forms that can infiltrate cells by unregulated routes and raise LCI to toxic levels. Despite the limited knowledge available on LPI speciation in different types and degrees of iron overload, LPI measurements can be and are in fact used for identifying systemic IO and for initiating/adjusting chelation regimens to attain full-day LPI protection. A recent application of labile iron assay is the detection of labile components in iv iron formulations per se as well as in plasma (LPI following parenteral iron

  19. Ectopic adrenal tissue of spermatic cord in a 3- year- old boy, an incidental finding during orchidopexy: A case report

    Biserka Pigac


    Full Text Available Ectopic adrenal tissue is usually found in kidney and retroperitoneum, yet other locations are considered uncommon. Spermatic cord remains one of the sites where this entity is mostly incidentally found during surgical procedure in childhood. Macroscopically, it represents as yellowish, lipomatous nodule. We present a case of ectopic adrenal tissue of spermatic cord in a 3- year- old boy, found during orchidopexy of the right testicle. Histopathological analysis of the spermatic cord nodule revealed adrenal cortical tissue composed of zona fasciculata cells. Although usually being benign and asymptomatic, cases of hyperplasia, adenoma and carcinoma arising from ectopic adrenal tissue have been reported. Therefore, removal of this tissue, when detected, is recommended.

  20. Lipofibromatosis: magnetic resonance imaging features and pathological correlation in three cases

    Vogel, Daniela; Righi, Alberto; Kreshak, Jennifer; Dei Tos, Angelo Paolo; Merlino, Biagio; Brunocilla, Eugenio; Vanel, Daniel


    Lipofibromatosis is a rare, benign, but infiltrative, soft tissue tumor seen in children. We present three cases of lipofibromatosis, each with different magnetic resonance imaging features and correlate this with the histological findings. The patients comprised two males and one female who presented in infancy; at birth, 5 months, and 7 months of age. Clinically, the masses were painless and slow-growing. The masses ranged in size from 2 to 6 cm and involved the distal extremities in two cases (one foot, one wrist) and the trunk. Magnetic resonance imaging showed lipomatous lesions with varying amounts of adipose and solid components in each case. There were no capsules at the periphery of the lesions. One case showed a fat-predominant lesion, another an equal mixture of fat and solid tissue, and the third was predominantly solid. This was reflected in the histology, which showed corresponding features. Radiological and histopathological differential diagnoses are reviewed. (orig.)

  1. Primary (Poorly Differentiated Sclerosing Liposarcoma of Temporal Region. An Uncommon Tumor in a Rare Site: A Case Report

    Anuradha CK Rao


    Full Text Available Liposarcoma (LS in the head and neck region is a rare tumor. The sclerosing variant of LS is a subtype of well-differentiated LS characterized by areas of conventional LS admixed with hypocellular areas of stromal sclerosis that show atypical lipomatous cells. The (poorly differentiated sclerosing LS, on the other hand, is more cellular with atypical, pleomorphic and often bizarre giant tumor cells admixed with atypical lipoblasts. We report a case of poorly differentiated sclerosing LS of temporal region in a 49-year-old man. Radiologically, the tumor was dumbbell shaped with intra and extra cranial extension. In this case, we discuss the clinico-radiological and pathological findings of an unusual tumor in a rare location. [J Interdiscipl Histopathol 2015; 3(1.000: 33-35

  2. Delineation of Chondroid Lipoma: An Immunohistochemical and Molecular Biological Analysis

    de Vreeze, Ronald S. A.; van Coevorden, Frits; Boerrigter, Lucie; Nederlof, Petra M.; Haas, Rick L.; Bras, Johannes; Rosenwald, Andreas; Mentzel, Thomas; de Jong, Daphne


    Aims. Chondroid lipoma (CL) is a benign tumor that mimics a variety of soft tissue tumors and is characterized by translocation t(11;16). Here, we analyze CL and its histological mimics. Methods. CL (n = 4) was compared to a variety of histological mimics (n = 83) for morphological aspects and immunohistochemical features including cyclinD1(CCND1). Using FISH analysis, CCND1 and FUS were investigated as potential translocation partners. Results. All CLs were strongly positive for CCND1. One of 4 myoepitheliomas, CCND1, was positive. In well-differentiated lipomatous tumors and in chondrosarcomas, CCND1 was frequently expressed, but all myxoid liposarcomas were negative. FISH analysis did not give support for direct involvement of CCND1 and FUS as translocation partners. Conclusions. Chondroid lipoma is extremely rare and has several and more prevalent histological mimics. The differential diagnosis of chondroid lipomas can be unraveled using immunohistochemical and molecular support. PMID:21559269

  3. Lipofibromatosis: magnetic resonance imaging features and pathological correlation in three cases

    Vogel, Daniela; Righi, Alberto; Kreshak, Jennifer; Dei Tos, Angelo Paolo [Istituto Ortopedico Rizzoli, Bologna (Italy); Merlino, Biagio [Universita Cattolica del Sacro Cuore Policlinico ' ' A. Gemelli' ' , Dipartimento di Scienze Radiologiche, Roma (Italy); Brunocilla, Eugenio [U.O. di UROLOGIA, Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Bologna (Italy); Vanel, Daniel [Istituto Ortopedico Rizzoli, Anatomia Patologica, Bologna (Italy)


    Lipofibromatosis is a rare, benign, but infiltrative, soft tissue tumor seen in children. We present three cases of lipofibromatosis, each with different magnetic resonance imaging features and correlate this with the histological findings. The patients comprised two males and one female who presented in infancy; at birth, 5 months, and 7 months of age. Clinically, the masses were painless and slow-growing. The masses ranged in size from 2 to 6 cm and involved the distal extremities in two cases (one foot, one wrist) and the trunk. Magnetic resonance imaging showed lipomatous lesions with varying amounts of adipose and solid components in each case. There were no capsules at the periphery of the lesions. One case showed a fat-predominant lesion, another an equal mixture of fat and solid tissue, and the third was predominantly solid. This was reflected in the histology, which showed corresponding features. Radiological and histopathological differential diagnoses are reviewed. (orig.)

  4. Mammary type myofibroblastoma of the buttock: A case report and review of the literature

    Park, Jong Min; Yi, Jae Hyuck [Dept.of Radiology, Kyungpook National University Hospital, College of Medicine, Kyungpook National University, Daegu (Korea, Republic of)


    Mammary-type myofibroblastoma is a very rare, benign mesenchymal tumor consisting of spindle-shaped cells along with thick hyalinized collagen bundles and an intralesional fat component; its histopathological features are identical to those of myofibroblastomas of the breast. It usually occurs along the embryonic milk-line; however, unusual cases occurring outside of the embryonic milk-line have also been reported. Although this tumor always shows clinically benign behavior, its variable histological composition can easily be confused with many other fibrous and lipomatous neoplasms. Unfortunately, its radiological findings are extremely rarely described in the literature. Here, we present a rare case of mammary-type myofibroblastoma in a 38-year-old woman who presented with a well-circumscribed solitary mass in the buttock, and discuss various radiologic imaging findings, such as plain radiography, ultrasonography, and magnetic resonance imaging results.

  5. Characterization of myocardial T1-mapping bias caused by intramyocardial fat in inversion recovery and saturation recovery techniques

    Kellman, Peter; Bandettini, W Patricia; Mancini, Christine


    BACKGROUND: Quantitative measurement of T1 in the myocardium may be used to detect both focal and diffuse disease processes such as interstitial fibrosis or edema. A partial volume problem exists when a voxel in the myocardium also contains fat. Partial volume with fat occurs at tissue boundaries...... imaging protocols using balanced steady state free precession are considered. In-vivo imaging with T1-mapping, water/fat separated imaging, and late enhancement imaging was performed on subjects with chronic myocardial infarction. RESULTS: In n = 17 subjects with chronic myocardial infarction, lipomatous...... agreement with simulation of the specific imaging protocols. CONCLUSIONS: Measurement of the myocardial T1 by widely used balanced steady state free precession mapping methods is subject to bias when there is a mixture of water and fat in the myocardium. Intramyocardial fat is frequently present...

  6. Pituitary adenoma with adipose tissue: A new metaplastic variant.

    Caporalini, Chiara; Buccoliero, Anna Maria; Pansini, Luigi; Moscardi, Selene; Novelli, Luca; Baroni, Gianna; Bordi, Lorenzo; Ammannati, Franco; Taddei, Gian Luigi


    Pituitary adenomas are benign tumors representing approximately 15-20% of intracranial neoplasms. There have been few reports of metaplastic osseous transformation and about 60 cases of neuronal metaplasia in pituitary adenoma but adipose metaplasia has not been previously described in the English literature. Here we report a case of pituitary adenoma with metaplastic adipose tissue in a 58-year-old male patient. Histologically this case fulfilled the criteria of a non-functioning pituitary adenoma, and moreover a central area of adipose tissue, made by mature adipocytes, and many tumor cells, containing fat droplet were evident. Lipomatous transformation of tumor cells in the CNS has been previously observed but, to the best of our knowledge, our case is the first pituitary adenoma with such change. The histogenesis of the adipose element in pituitary adenoma is not well understood, and could be a result of a metaplastic change or divergent differentiation from a common progenitor cell. © 2017 Japanese Society of Neuropathology.

  7. Ossifying chondrolipoma of the tongue

    Tasić Desanka


    Full Text Available Introduction. Chondrolipomas and osteolipomas are uncommon variants of lipomatous tumors. Case report. We presented a 60-year-old woman with ossifying chondrolipoma of the tongue. Clinical examination revealed a firm nodular mass, located in the midline of the posterior region on the dorsal surface of the tongue. Histologically, the lesion was well-delimited showing areas of mature adipocytes arranged in lobules and separated by fibrous connective tissue septa, islands of mature cartilaginous tissue and osseous metaplasia. Trabeculae of lamellar bone within a fibro-fatty background were visible throughout the tumor. The cartilaginous areas merging centrally with bone formation and fatty marrow tissue were present, as well as the hematopoietic elements in the fatty marrow. The bone forming was found to be through both membranous and enchondral mechanisms. Conclusion. Ossifying chrondrolipoma with hematopoietic elements is extremely unusual lesion. This interesting entity should be kept in mind in the differential diagnosis of lingual lesions.

  8. Liposarcoma of soft tissue: MRI findings with pathologic correlation

    Arkun, R.; Memis, A.; Ustun, E.E.; Akalin, T.; Kandiloglu, G.; Sabah, D.


    The MR images of seven liposarcomas were reviewed retrospectively to assess the tumor size, location, margination, signal characteristics and enhancement patterns in differnt histologic types. Seven liposarcomas comprising three well-differntiated, two myxoid and two pleomorphic types were evaluated. All tumors showed well-defined and mostly lobulated margins. The well-differentiated liposarcomas were composed mainly of fat with septations or nodules, were hyperintense on T2-weighted images, and demonstrated faint enhancement or no enhancement following intravenous constrast. Myxoid liposarcomas were homogeneous or mildly heterogenous and a pseudocapsule was present in one case. Pleomorphic tyes showed a markedly heterogeneous internal structure. Both myxoid and pleomorphic lesions-showed moderate or marked heterogeneous enhancement after contrast administration. Well-differentiated liposarcomas may by differntiated from other types of the tumor by their largely lipomatous apperance. The malignancy grade increases in parallel with tumor heterogeneity and contrast enhancement. (orig.)

  9. Transoral robotic assisted resection of the parapharyngeal space.

    Mendelsohn, Abie H


    Preliminary case series have reported clinical feasibility and safety of a transoral minimally invasive technique to approach parapharyngeal space masses. With the assistance of the surgical robotic system, tumors within the parapharyngeal space can now be excised safely without neck incisions. A detailed technical description is included. After developing compressive symptoms from a parapharyngeal space lipomatous tumor, the patient was referred by his primary otolaryngologist because of poor open surgical access to the nasopharyngeal component of the tumor. Transoral robotic assisted resection of a 54- × 46-mm parapharyngeal space mass was performed, utilizing 97 minutes of robotic surgical time. Pictorial demonstration of the robotic resection is provided. Parapharyngeal space tumors have traditionally been approached via transcervical skin incisions, typically including blunt dissection from tactile feedback. The transoral robotic approach offers magnified 3D visualization of the parapharyngeal space that allows for complete and safe resection. © 2014 Wiley Periodicals, Inc.

  10. Imaging findings of adiposis dolorosa vs. massive localized lymphedema

    Petscavage-Thomas, Jonelle M.; Bernard, Stephanie A.; Bennett, Jennifer [Milton S. Hershey Medical Center, Department of Radiology, H066, 500 University Drive, P.O. Box 850, Hershey, PA (United States); Walker, Eric A. [Milton S. Hershey Medical Center, Department of Radiology, H066, 500 University Drive, P.O. Box 850, Hershey, PA (United States); Uniformed Services University of the Health Sciences, Department of Radiology and Nuclear Medicine, Bethesda, MD (United States)


    Adiposis dolorosa (Dercum's disease) is a condition of benign, painful subcutaneous lipomatous lesions associated with weakness, endocrine and lipid abnormalities, and mental disturbances. There is little information documenting the cross-sectional imaging findings that differentiate it from lipomatous and neoplastic soft tissue masses, or massive localized lymphedema. The purpose of this study was to provide a radiological case series of adiposis dolorosa. A 10-year retrospective review of the picture archiving and communications system was performed. Two musculoskeletal radiologists reviewed images to confirm and document imaging features, location, size, and patient demographics. Medical records were reviewed to characterize patients into three groups: one group met at least three of the four criteria of Dercum's syndrome, the second group met less than three criteria, and the third group had clinical diagnosis of cellulitis of the lower extremity. Seventeen cases (25 masses) of adiposis dolorosa were found, nine cases of which met at least three criteria of Dercum's syndrome. All cases in the first two groups demonstrated skin thickening and lymphedema of subcutaneous fat, which was fluid attenuation on CT and low or intermediate T1-weighted and high STIR/T2-weighted MR signal. Two cases with pathology showed mild fatty infiltration with fibrous septa, and the third case showed massive localized lymphedema. The third group of ten cellulitis patients demonstrated non-mass-like subcutaneous edema with similar CT attenuation and MR signal characteristics to the first two groups, but differed by the presence of post-contrast enhancement and non-mass-like appearance in 90 %. Imaging findings of adiposis dolorosa and massive localized lymphedema overlap, as do the symptoms and pathological features. Due to the mass-like engorgement of the soft tissues and pain, patients will often undergo imaging to exclude neoplasm or infection. Knowledge of these

  11. Cerebellar liponeurocytoma: a newly recognized clinico-pathological entity Liponeurocitoma cerebelar: uma nova entidade clínico-patológica

    Nádia Montagna


    Full Text Available The term "cerebellar liponeurocytoma", recently adopted by the World Health Organization Working Group (WHO, replaced many other different terms used up to now to give name to this rare tumor. To our knowledge, less than 20 cases have been related up to now under different names like as "lipomatous medulloblastoma, lipidized medulloblastoma, neurolipocytoma, medullocytoma and lipomatous glioneurocytoma". The new nomenclature eliminates the word "medulloblastoma", reinforces its benign caracter, and includes it in the category of glioneuronal tumors. We describe an adictional case of this distinct clinico-pathological entity removed from the right cerebellar hemisphere of a 53-year-old woman. With the present case report, we hope to contribute to the knowledge on the diagnostic and prognostic implications derived from the finding of mature adipose-like tissue within a medulloblastomatous tumour.O termo "liponeurocitoma cerebelar" recentemente adotado pela Organização Mundial de Saúde (Classificação de Tumores Cerebrais - versão 2000, surgiu em substituição a vários outros utilizados até então, para denominar esta rara neoplasia. De nosso conhecimento há na literatura menos de 20 casos relatados sob termos diferentes tais como "meduloblastoma lipomatoso, meduloblastoma lipidizado, neurolipocitoma, medulocitoma e glioneurocitoma lipomatoso". A nova nomenclatura elimina a palavra "meduloblastoma", enfatiza seu caráter benigno e o coloca na categoria dos tumores glioneuronais. Descrevemos mais um caso desta rara entidade clínico-patológica, ressecada do hemisfério cerebelar direito em uma mulher de 53 anos. Com este caso esperamos contribuir para o melhor conhecimento sobre o diagnóstico, prognóstico e possibilidades terapêuticas advindas da presença de tecido adiposo em tumor meduloblastomatoso de adultos.

  12. Histological spectrum of angiofibroma of soft tissue: histological and genetic analysis of 13 cases.

    Yamada, Yuichi; Yamamoto, Hidetaka; Kohashi, Kenichi; Ishii, Takeaki; Iura, Kunio; Maekawa, Akira; Bekki, Hirofumi; Otsuka, Hiroshi; Yamashita, Kyoko; Tanaka, Hiroyuki; Hiraki, Tsubasa; Mukai, Munenori; Shirakawa, Atsuko; Shinnou, Yoko; Jinno, Mari; Yanai, Hiroyuki; Taguchi, Kenichi; Maehara, Yoshihiko; Iwamoto, Yukihide; Oda, Yosinao


    Angiofibroma of soft tissue (AFST) is a rare soft tissue neoplasm characterized by a fibroblastic cytomorphology and a prominent vascular structure. AFSTs possess a novel fusion gene, i.e. NCOA2-AHRR/AHRR-NCOA2 or GTF2I-NCOA2, providing a useful approach to diagnosing AFST. Morphologically, AFSTs span a wide spectrum, making diagnosis a challenge. The aim of this study was to review AFST cases and to report previously unknown histological features, which we confirmed by genetic analysis. We reviewed 276 cases diagnosed as solitary fibrous tumours/haemangiopericytomas (232 cases), unclassified tumours of fibroblastic differentiation (36 cases), and recently diagnosed AFSTs (eight cases), and retrieved 13 cases compatible with AFST. Immunohistochemical staining was performed for these cases, all 13 of which were analysed by reverse transcription polymerase chain reaction and fluorescence in-situ hybridization. The histological findings were as follows: amianthoid fibres, extravasation of red blood cells, haemosiderin deposition, aggregates of foamy histiocytes, cystic change, necrosis, and haemorrhage. Immunohistochemically, the tumour cells were positive for epithelial membrane antigen (four of 13 cases), desmin (six of 13 cases), CD163 (13 of 13 cases), CD68 (seven of 13 cases), oestrogen receptor (13 of 13 cases), progesterone receptor (three of 13 cases), and STAT6 (one of 13 cases, weak nuclear staining), but they were negative for CD34, α-smooth muscle actin, muscle-specific actin, S100, pan-cytokeratin, MDM2, and CDK4. The AHRR-NCOA2 fusion gene was detected in eight cases, and NCOA2 gene rearrangement in nine cases. We revealed the previously unreported histological variation and immunohistochemical findings of AFST, and confirmed them by using genetic methods. The results suggested that AFST should be considered in the diagnosis of fibrous or fibrohistiocytic tumours with the above histological features. © 2016 John Wiley & Sons Ltd.

  13. A 20 year retrospective histomorphological analysis of juvenile soft tissue tumors

    Odokuma Emmanuel Igho


    Full Text Available Introduction: Soft tissue tumours (STT are defined traditionally as mesenchymal proliferations that occur in the extra-.skeletal nonepithelial tissues of the body excluding viscera, meninges and lymphoreticular system. These tumours occur in children where they may result in severe debilitating disease. This study was therefore aimed at determining the age, gender and site distribution of soft tissue tumours in the young. Materials and Methods: The records of all pathology consultations during the 20 year period from (1990-2010, from the Department of Morbid Anatomy/Histopathology, University of Benin Teaching Hospital from birth to 20 years, were utilized for this study. The lesions were standardized in accordance with the world health organization (WHO classification. Permission for this study was obtained from the UBTH ethics committee (protocol number ADM/E22/A/VOL.VII/142. Results: A total of 139 lesions were recorded, 72 males and 67 females with a male/female ratio of 1.1:1. Benign tumours constituted 113(81% while malignant tumours accounted for 26(19%. This study demonstrated that, nerve sheath tumours and vascular tumours accounted for 25% of STT in children followed by adipocytic tumours 22%, skeletal muscle tumours 17%, fibrohistiocytic tumours 7%, fibroblastic tumours 5%, and perivascular tumours 1% respectively. Majority of these tumours were located in the head and lower extremities with fewer in the upper extremities and trunk. These lesions were predominant in females of the older age group (10-20 years unlike in their male counterparts. Conclusion: This study has shown that benign soft tissue tumours are more prevalent than the malignant varieties in juveniles.

  14. Cellular angiofibroma with atypia or sarcomatous transformation: clinicopathologic analysis of 13 cases.

    Chen, Eleanor; Fletcher, Christopher D M


    Cellular angiofibroma is a mesenchymal neoplasm that is characterized by a bland spindle cell component, morphologically reminiscent of spindle cell lipoma, and thick-walled vessels. The tumor occurs equally in men and women and usually arises in the inguino-scrotal or vulvovaginal regions. An earlier study of 51 cases from our group showed that the tumor follows a benign course without any tendency for recurrence. In 1 case, an intralesional microscopic nodule of pleomorphic liposarcoma was observed. The biologic significance of atypia or sarcomatous transformation in cellular angiofibroma remains uncertain. In this study, we characterized clinicopathologic features in 13 cases of cellular angiofibroma with morphologic atypia or sarcomatous transformation. Thirteen cases with atypia or sarcomatous transformation among 154 usual cellular angiofibromas identified between 1993 and 2009 were retrieved from consultation files. There were 12 females and 1 male ranging in age from 39 to 71 years (median age, 46 y). Tumor size ranged from 1.2 to 7.5 cm. In 11 cases, the tumors occurred in the vulva. One case each occurred in the paratesticular and hip regions. Most tumors were located in subcutaneous tissue. There were 4 cases of cellular angiofibroma with atypia. Three showed severely atypical cells as scattered foci within the cellular angiofibroma. One case showed a discrete nodule of atypical cells. There were 9 cases of cellular angiofibroma with morphologic features of sarcomatous transformation. In each case, abrupt transition to a discrete sarcomatous component was seen. Of these 9 cases, the sarcomatous component in 2 cases showed features of pleomorphic liposarcoma with multivacuolated lipoblasts readily identified. Three of these 9 cases showed discrete nodule(s) closely resembling atypical lipomatous tumor within usual cellular angiofibroma. In the remaining 4 cases, the sarcomatous component was composed of pleomorphic spindle cells arranged in various

  15. Bony sequestrum: A radiologic review

    Jennin, Felicie; Bousson, Valerie; Parlier, Caroline; Jomaah, Nabil; Khanine, Vanessa; Laredo, Jean-Denis [Lariboisiere Hospital, Department of Radiology, Paris (France)


    According to a pathological definition, a bony sequestrum is defined as a piece of devitalized bone that has been separated from the surrounding bone during the process of necrosis. However, the radiological definition of a sequestrum is different and refers to an image of calcification visible within a lucent lesion, completely separated from the surrounding bone, without referring to the vascular status and histological nature of the calcified tissue. The term ''button sequestrum'' has been used in calvarial lesions. The prototype conditions that may present with a bony sequestrum are osteomyelitis and skeletal tuberculosis. Other conditions such as radiation necrosis, eosinophilic granuloma, metastatic carcinoma, primary lymphoma of bone, aggressive fibrous tumors may also manifest as osteolytic lesions containing a sequestrum. In addition, some primary bone tumors produce a matrix that may mineralize and sometimes simulate a bone sequestrum. These include osteoid tumors (osteoid osteoma, osteoblastoma), cartilaginous tumors (chondroma and chondroblastoma), lipomatous tumors (lipoma), and benign fibrous tumors (fibromyxoma, myxoma, and desmoplastic fibroma). Therefore, various conditions may present at imaging as a small area of osteolysis containing central calcifications. However, a careful analysis of the sequestrum as well as the associated clinical and radiological findings often enables to point toward a limited number of conditions. (orig.)

  16. Fine needle aspiration biopsy diagnosis of dedifferentiated liposarcoma: Cytomorphology and MDM2 amplification by FISH

    Al-Maghraby Hatem


    Full Text Available Lipomatous mesenchymal tumors constitute the most common type of soft tissue tumors. Well-differentiated liposarcoma (WDLS can undergo dedifferentiation to a nonlipogenic sarcoma of variable histologic grade. In the recent literature, amplification of the murine double minute 2 (MDM2 oncogene, which has a role in cell cycle control, has been successful in distinguishing WDLS from benign lesions. We present a case of dedifferentiated liposarcoma diagnosed by fine-needle aspiration (FNA, using cytomorphology and ancillary studies (immunocytochemistry and fluorescent in-situ hybridization. An 85-year old female presented to our institution with a firm soft tissue mass of the right buttock. The FNA showed atypical spindle cells, osteoclast-like giant cells and extracellular dense matrix material. The cell block showed cellular groups of highly atypical spindle cells with osteoid and adipose tissue. Fluorescence in situ hybridization (FISH studies performed on the cell block demonstrated amplification of the MDM2 gene. In addition, the findings were morphologically compatible with the previously resected retroperitoneal dedifferentiated liposarcoma with areas of osteosarcoma. This rare case illustrates the usefulness of FNA and ancillary studies in the diagnosis and subclassification of soft tissue tumors. To the best of our knowledge, this is the first report of MDM2 FISH positivity in a liposarcoma diagnosed by FNA.

  17. Contributions of Cytogenetics and Molecular Cytogenetics to the Diagnosis of Adipocytic Tumors

    Jun Nishio


    Full Text Available Over the last 20 years, a number of tumor-specific chromosomal translocations and associated fusion genes have been identified for mesenchymal neoplasms including adipocytic tumors. The addition of molecular cytogenetic techniques, especially fluorescence in situ hybridization (FISH, has further enhanced the sensitivity and accuracy of detecting nonrandom chromosomal translocations and/or other rearrangements in adipocytic tumors. Indeed, most resent molecular cytogenetic analysis has demonstrated a translocation t(11;16(q13;p13 that produces a C11orf95-MKL2 fusion gene in chondroid lipoma. Additionally, it is well recognized that supernumerary ring and/or giant rod chromosomes are characteristic for atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma, and amplification of 12q13–15 involving the MDM2, CDK4, and CPM genes is shown by FISH in these tumors. Moreover, myxoid/round cell liposarcoma is characterized by a translocation t(12;16(q13;p11 that fuses the DDIT3 and FUS genes. This paper provides an overview of the role of conventional cytogenetics and molecular cytogenetics in the diagnosis of adipocytic tumors.

  18. Fibrolipomatous hamartoma: pathognomonic on MR imaging

    Marom, E.M.; Helms, C.A.


    Purpose. To assess the MR imaging characteristics, presenting symptoms, age and nerve distribution of fibrolipomatous hamartoma. Design. A computer search was performed of the term fibrolipomatous hamartoma through the musculoskeletal section MR imaging results at our institution from June 7, 1996 to January 21, 1998 followed by a search of the terms lipomatous hamartoma, median nerve, surrounding fat, increased fatty signal, coaxial, and neuroma. MR images and medical files were retrospectively reviewed by two experienced musculoskeletal radiologists for imaging characteristics, nerve and age distribution as well as for history of trauma. In addition three consultation cases from outside institutions were added for determination of image characteristics. Results. Ten fibrolipomatous hamartomas were identified: eight in the median nerve, one in the ulnar nerve and one in the sciatic nerve. Mean age was 32.3 years (range 4-75 years, SD 21 years). Imaging characteristics were serpiginous low-intensity structures representing thickened nerve fascicles, surrounded by evenly distributed fat, high signal intensity on T1-weighted sequences and low signal intensity on T2-weighted sequences. The amount of fat varied; however, distribution in eight cases (80%) was predominantly between nerve fibers rather than surrounding them peripherally. All had a coaxial-cable-like appearance on axial planes and a spaghetti-like appearance on coronal planes that was not seen in any other type of median nerve abnormality imaged during the study period. Conclusion. The MR imaging characteristics of fibrolipomatous hamartoma are pathognomonic, obviating the need for biopsy for diagnosis. (orig.)

  19. [Mediastinal teratoma with malignant transformation of the somatic component. Clinical report].

    Gerardo, Rita; Morgado, Carolina; Calvo, Dolores; Pinto, Eugénia; Bravio, Ivan; Castelão, Nelson; Martelo, Fernando


    Mediastinal germ cell tumours (M-GCT) are rare forms of neoplasms compared with other tumours of the same location. They are classified in seminomas, malignant non-seminomatous GCT and teratomas. The malignant transformation of the somatic component of the teratoma, with sarcomatous or carcinomatous degeneration, is even more uncommon. We report the clinical case of a 32 year old man who presented with severe chest pain on the right hemithorax. The image exams revealed the existence of a large heterogeneous lesion with a diameter of 7.7 cm, with areas of lipomatous density and a calcic image with the appearance of a tooth, in the right projection of the anterior mediastinum, in the vicinity of the large vessels, compatible with teratoma. The transthoracic biopsy (CT guided) showed morphologic aspects of sarcoma. The patient was operated on with the en bloc resection of the mediastinal mass, right lung, a segment of the pericardium and the thymus. The pathological studies showed a teratoma with malignant transformation of the mesenquimatous component, with muscular differentiation into leiomiosarcoma and rabdomiosarcoma. After surgery, the patient was treated with a scheme of doxorubicin and ifosfamide. The most prominent concepts related to this clinical entity, as well as its treatment, are debated in this article, based on the most recent publications dedicated to the subject.

  20. Transdiaphragmatic extension of a retroperitoneal lipoma into the intrathoracic extrapleural space via the lumbocostal trigone in a dog.

    Klosterman, Emily S; Heng, Hock Gan; Freeman, Lynetta J; Childress, Michael O


    An 8-year-old 12.2-kg (26.9-lb) spayed female American Cocker Spaniel was referred for evaluation of abdominal discomfort and a suspected intra-abdominal lipoma with possible invasion into the thorax. Physical examination revealed a tense abdomen, and the margins of a large abdominal mass could be appreciated. Abdominal imaging revealed a mass of fat opacity in the abdominal and thoracic cavities. Computed tomography with precontrast and postcontrast peritoneograms was used to determine whether the masses connected via a diaphragmatic defect. Exploratory laparotomy revealed a retroperitoneal lipomatous mass that had focally invaded the hypaxial musculature and had extended across the dorsolateral aspect of the diaphragm via the lumbocostal trigone into the intrathoracic extrapleural space. Surgical resection required transdiaphragmatic thoracotomy. Histologic examination of excised tissue confirmed the preoperative diagnosis of a lipoma. The dog recovered from surgery with no complications and had no disease recurrence for at least 32 months after surgical resection. The defect of the lumbocostal trigone is also called the foramen of Bochdalek in humans, and it is recognized as a common location for congenital diaphragmatic hernia. A lumbocostal trigone hernia may be considered as a differential diagnosis for bicavitary masses in dogs, particularly in the absence of a history of trauma.

  1. Comparison of Chromogenic In Situ Hybridization and Fluorescence In Situ Hybridization for the Evaluation of MDM2 Amplification in Adipocytic Tumors.

    Mardekian, Stacey K; Solomides, Charalambos C; Gong, Jerald Z; Peiper, Stephen C; Wang, Zi-Xuan; Bajaj, Renu


    Atypical lipomatous tumor/well-differentiated liposarcoma (ALT-WDLPS) and dedifferentiated liposarcoma (DDLPS) are characterized cytogenetically by a 12q13-15 amplification involving the mouse double minute 2 (MDM2) oncogene. Fluorescence in situ hybridization (FISH) is used frequently to detect this amplification and aid with the diagnosis of these entities, which is difficult by morphology alone. Recently, bright-field in situ hybridization techniques such as chromogenic in situ hybridization (CISH) have been introduced for the determination of MDM2 amplification status. The present study compared the results of FISH and CISH for detecting MDM2 amplification in 41 cases of adipocytic tumors. Amplification was defined in both techniques as a MDM2/CEN12 ratio of 2 or greater. Eleven cases showed amplification with both FISH and CISH, and 26 cases showed no amplification with both methods. Two cases had discordant results between CISH and FISH, and two cases were not interpretable by CISH. CISH is advantageous for allowing pathologists to evaluate the histologic and molecular alterations occurring simultaneously in a specimen. Moreover, CISH is found to be more cost- and time-efficient when used with automation, and the signals do not quench over time. CISH technique is a reliable alternative to FISH in the evaluation of adipocytic tumors for MDM2 amplification. © 2014 Wiley Periodicals, Inc.

  2. MRI features of spinal epidural angiolipomas

    Hu, Su; Hu, Chun Hong; Wang, Xi Ming; Dai, Hui [Dept. of Radiology, The First Affiliated Hospital of Soochow University, Jiangsu (China); Hu, Xiao Yun; Fang, Xiang Ming [Dept. of Radiology, Wuxi People' s Hospital Affiliated to Nanjing Medical University, Jiangsu (China); Cui, Lei [Dept. of Radiology, The Second Affiliated Hospital of Nantong University, Jiangsu (China)


    To describe the MRI findings in ten patients of spinal epidural angiolipoma for differentiated diagnosis presurgery. Ten surgically proved cases of spinal epidural angiolipomas were retrospectively reviewed, and the lesion was classified according to the MR findings. Ten tumors were located in the superior (n = 4), middle (n = 2), or inferior (n = 4) thoracic level. The mass, with the spindle shape, was located in the posterior epidural space and extended parallel to the long axis of the spine. All lesions contained a fat and vascular element. The vascular content, correlating with the presence of hypointense regions on T1-weighted imaging (T1WI) and hyperintense signals on T2-weighted imaging, had marked enhancement. However, there were no flow void signs on MR images. All tumors were divided into two types based on the MR features. In type 1 (n = 3), the mass was predominantly composed of lipomatous tissue (> 50%) and contained only a few small angiomatous regions, which had a trabeculated or mottled appear. In type 2 (n = 7), the mass, however, was predominantly composed of vascular components (> 50%), which presented as large foci in the center of the mass. Most spinal epidural angiolipomas exhibit hyperintensity on T1WI while the hypointense region on the noncontrast T1WI indicates to be vascular, which manifests an obvious enhancement with gadolinium administration.

  3. Angiolipoma involving the spine: a report of two cases and a review of the literature

    Munoz, M. M.; Montero, M. J.; Garcia de la Oliva, T.; Ballenilla, L.; Palacios, S.


    Angiolipoma of the spinal column is a lesion made up of mature adipocytes with abnormal blood vessels. Around 60 cases have been reported, the majority located in the extradural space, predominantly the dorsal area. We report two cases examined in our hospital by means of plain X ray, computed tomography and magnetic resonance (MR) imaging. We review the literature and the findings with the different imaging techniques, stressing the importance of the use of MR, especially those techniques involving fat suppression, in the preoperative diagnosis of these lesions. In general, they present high signal intensity on T1-weighted images, with some areas of iso/hyposignal in the interior, a finding that differentiates them from lipomatosis, a condition that presents high signal intensify on T1-weighted sequences, but homogeneously. The administration of a paramagnetic contrast agent enhanced the images, but to a lesser extent in lesions with very high signal intensity on T1-weighted images. The signal intensity can be variable or high on T2-weighted sequences. Techniques using fat suppression are especially specific since they enhance the non-lipomatous (angiomatous) component of the lesion. The association of these MR findings with adjacent skeletal changes, consisting of progressive spinal cord compression, should lead to a suspicion of this un-common lesion, which is successfully managed with surgery. (Author) 14 refs

  4. CT characteristics of primary retroperitoneal neoplasms in children

    Xu Yufeng; Wang Jichen; Peng Yun; Zeng Jinjin


    Primary retroperitoneal neoplasms are uncommon in children. Retroperitoneal neoplasms are either mesodermal, neurogenic, germ cell ectodermal or lymphatic in origin. In general, primary retroperitoneal neoplasms in children have different spectrum and prevalence compared to those in adults. Neuroblastoma, rhabdomyosarcoma, benign teratoma and lymphoma are the common retroperitoneal neoplasms. In this review, the clinical and CT futures of common retroperitoneal neoplasms in children are described. Coarse, amorphous, and mottled calcification are very common in neuroblastoma. Paraganglioma tends to show marked and early enhancement and may present with clinical symptoms associated with the excess catecholamine. Sarcomas are often very large and have heterogeneous appearance. Imaging cannot be reliably used to identify the type of retroperitoneal sarcomas due to overlapped radiographic features. In children, lipoblastoma is the most common lipomatous tumor in the retroperitoneum. The percentage of visible fat in tumor varies depending on the cellular composition of the lesion. The CT characteristics of teratoma are quite variable, which may be cystic, solid, on a combination of both. Typically teratoma appears as a large complex mass containing fluid, fat, fat-fluid level, and calcifications. Lymphoma is often homogeneous on both enhanced and unenhanced CT scans. Necrosis and calcification are rare on CT. In conclusion, making a final histological diagnosis of retroperitoneal tumor base on CT features is not often possible; however, CT can help to develop a differential diagnosis and determine the size and extent of the retroperitoneal neoplasms.

  5. A case of junctional neural tube defect associated with a lipoma of the filum terminale: a new subtype of junctional neural tube defect?

    Florea, Simona Mihaela; Faure, Alice; Brunel, Hervé; Girard, Nadine; Scavarda, Didier


    The embryological development of the central nervous system takes place during the neurulation process, which includes primary and secondary neurulation. A new form of dysraphism, named junctional neural tube defect (JNTD), was recently reported, with only 4 cases described in the literature. The authors report a fifth case of JNTD. This 5-year-old boy, who had been operated on during his 1st month of life for a uretero-rectal fistula, was referred for evaluation of possible spinal dysraphism. He had urinary incontinence, clubfeet, and a history of delayed walking ability. MRI showed a spinal cord divided in two, with an upper segment ending at the T-11 level and a lower segment at the L5-S1 level, with a thickened filum terminale. The JNTDs represent a recently classified dysraphism caused by an error during junctional neurulation. The authors suggest that their patient should be included in this category as the fifth case reported in the literature and note that this would be the first reported case of JNTD in association with a lipomatous filum terminale.

  6. The right atrium: gateway to the heart--anatomic and pathologic imaging findings.

    Malik, Sachin B; Kwan, Damon; Shah, Amar B; Hsu, Joe Y


    Knowledge of right atrial anatomic and pathologic imaging findings and associated clinical symptoms is important to avoid false-positive diagnoses and missed findings. Complete evaluation of the heart often requires a multimodality approach that includes radiography, echocardiography, computed tomography (CT), magnetic resonance (MR) imaging, and invasive angiography. In general, CT provides the highest spatial resolution of these modalities at the cost of radiation exposure to the patient. Echocardiography and MR imaging offer complementary and detailed information for functional evaluation without added radiation exposure. The advantages and disadvantages of each modality for the evaluation of right atrial anatomic structure, size, and pathologic findings are discussed. Cardiac MR imaging is the reference standard for evaluation of right atrial size and volume but often is too time consuming and resource intensive to perform in routine clinical practice. Therefore, established reference ranges for two-dimensional transthoracic echocardiography are often used. Right atrial pathologic findings can be broadly categorized into (a) congenital anomalies (cor triatriatum dexter, Ebstein anomaly, and aneurysm), (b) disorders of volume (tricuspid regurgitation, pathologic mimics such as a pseudoaneurysm, and atrial septal defect), (c) disorders of pressure (tricuspid stenosis, restrictive cardiomyopathy, and constrictive pericarditis), and (d) masses (pseudomasses, thrombus, lipomatous hypertrophy of the interatrial septum, lipoma, myxoma, sarcoma, and metastatic disease). Familiarity with each pathologic entity and its treatment options is essential to ensure that appropriate imaging modalities are selected. Online supplemental material is available for this article. RSNA, 2015

  7. Pleomorphic malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the scrotum in a patient presenting as fournier gangrene: a case report.

    Guo, Juan; Zhou, Shengmei; Rao, Nagesh P; Pez, Gholam H


    Pleomorphic malignant fibrous histiocytoma (MFH), also known as undifferentiated high-grade pleomorphic sarcoma according to the latest World Health Organization classification, is a diagnosis of exclusion and extremely rare in adult scrotal/paratesticular region. Clinical presentation of scrotal/paratesticular pleomorphic MFH is usually a painless and gradual scrotal swelling. We report a case of scrotal MFH in a 63-year-old man who presented as Fournier gangrene after 10-month painful scrotal swelling and multiple procedures. The specimen of emergent debridement was submitted for pathologic and bacteriologic examination. Microscopically, the lesion had marked architectural and cytologic pleomorphism. The neoplastic cells were positive for vimentin, but negative for all lineage-specific markers. Fluorescence in-situ hybridization showed an aneuploid karyotype and negative results for lipomatous tumor abnormalities. Bacterial cultures of the specimen showed extensive growth of virulent polymicrobes. The diagnosis of scrotal/paratesticular pleomorphic MFH with concurrent Fournier gangrene was made. Thoracic computed tomography scan showed bilateral multiple pulmonary nodules. The patient died 1 month later.

  8. An overview on development and application of an experimental platform for quantitative cardiac imaging research in rabbit models of myocardial infarction.

    Feng, Yuanbo; Bogaert, Jan; Oyen, Raymond; Ni, Yicheng


    To exploit the advantages of using rabbits for cardiac imaging research and to tackle the technical obstacles, efforts have been made under the framework of a doctoral research program. In this overview article, by cross-referencing the current literature, we summarize how we have developed a preclinical cardiac research platform based on modified models of reperfused myocardial infarction (MI) in rabbits; how the in vivo manifestations of cardiac imaging could be closely matched with those ex vivo macro- and microscopic findings; how these imaging outcomes could be quantitatively analyzed, validated and demonstrated; and how we could apply this cardiac imaging platform to provide possible solutions to certain lingering diagnostic and therapeutic problems in experimental cardiology. In particular, tissue components in acute cardiac ischemia have been stratified and characterized, post-infarct lipomatous metaplasia (LM) as a common but hardly illuminated clinical pathology has been identified in rabbit models, and a necrosis avid tracer as well as an anti-ischemic drug have been successfully assessed for their potential utilities in clinical cardiology. These outcomes may interest the researchers in the related fields and help strengthen translational research in cardiovascular diseases.

  9. Muscle fibers inside a fat tumor: A non-specific imaging finding of benignancy

    Donato, M.; Vanel, D.; Alberghini, M.; Mercuri, M.


    Introduction: The differential diagnosis between benign and low-grade well-differentiated malignant lipomatous tumors might be very difficult for both the radiologist and the pathologist, although it has practical consequences. Among the criteria, muscular fibers detected inside the lesion are considered radiologically and histologically as a reliable sign of a benign intramuscular lipoma. New genetic criteria are now available. We report two cases of fat tumors containing muscular fibers both radiologically and histologically, but which are definitely malignant, considering genetic criteria. Material and methods: Two cases of soft tissue fat tumors, containing muscular fibers on imaging examinations as well as histologically, had an aggressive behaviour, suggesting malignancy. Genetic criteria were therefore used to confirm the clinical impression. Results: MDM2 and CDK4 confirmed the malignancy in the two cases. Conclusion: Intra lesional muscular fibers detected on imaging or histological examinations should not be considered as a completely reliable sign of a benign intramuscular lipoma. In case of atypical clinical behaviour, genetic criteria should be used to prove the aggressiveness of the tumor.

  10. New observations concerning the interpretation of magnetic resonance spectroscopy of meningioma

    Yue, Qiang [University of Tsukuba, Department of Neurosurgery, Institute of Clinical Medicine, Tsukuba Science City, Ibaraki (Japan); West China Hospital of Sichuan University, Huaxi MR Research Center, Department of Radiology, Chengdu (China); Isobe, Tomonori [Kitasato University, Department of Medical Technology, School of Allied Health Sciences, Minato (Japan); Shibata, Yasushi; Kawamura, Hiraku; Yamamoto, Youhei; Takano, Shingo; Matsumura, Akira [University of Tsukuba, Department of Neurosurgery, Institute of Clinical Medicine, Tsukuba Science City, Ibaraki (Japan); Anno, Izumi [University of Tsukuba, Department of Radiology, Institute of Clinical Medicine, Tsukuba (Japan)


    This study was aimed to clarify some ambiguities in the interpretation of proton magnetic resonance spectroscopy (1H-MRS) of meningiomas. The cases of 31 meningioma patients (27 benign and 4 nonbenign meningiomas) that underwent single-voxel 1H-MRS (PRESS sequence, TR/TE = 2,000 ms/68, 136, 272 ms) were retrospectively analyzed. To verify the findings of in-vivo study, phantoms were measured, and pathological sections of 11 patients were reviewed. All meningiomas demonstrated increased choline and decreased creatine, except for a lipomatous meningioma that only displayed a prominent lipid (Lip) peak. Alanine (Ala) and lactate (Lac) coexisted in eight cases, indicating an alternative pathway of energy metabolism in meningiomas. They partially overlapped with each other and demonstrated a triplet-like spectral pattern, which was consistent with phantom study. Glutamine/glutamate (Glx) was helpful for the recognition of meningioma when Ala was absent. N-acetyl compounds(NACs) were observed in nine cases whose voxels were completely limited within the tumors, indicating that meningiomas might have endogenous NACs. Lac was indicative of an aggressive meningioma, although not always a nonbenign one. Lip not only represented micronecrosis in nonbenign meningiomas, but also reflected microcystic changes or fatty degeneration in benign meningiomas. 1H-MRS reflects some distinctive biochemical and pathological changes of meningiomas that might be misinterpreted. (orig.)


    Ana Maria Abreu Velez


    Full Text Available Introduction: In patients with autoimmune skin blistering diseases (ABDs, the diagnostic gold standard has classically been direct and indirect immunofluorescence (DIF and IIF, despite inherent technical problems of autofluorescence. Aim: We sought to overcome autofluorescence issues and compare the reliability of immunofluorescence versus immunohistochemistry (IHC staining in the diagnoses of these diseases. Methods: We tested via IHC for anti-human IgG, IgM, IgA, IgD, IgE, Kappa light chains, Lambda light chains, Complement/C3c, Complement/C1q, Complement/C3d, albumin and fibrinogen in 30 patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF, and 30 control biopsies from the endemic area. We also tested archival biopsies from patients with ABDs whose diagnoses were made clinically, histopathologically and by DIF/IIF studies from 2 independent dermatopathology laboratories in the USA. Specifically, we tested 34 patients with bullous pemphigoid (BP, 18 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus (PF, 14 with dermatitis herpetiformis (DH and 30 control skin samples from plastic esthetic surgery reduction surgeries. Results: The diagnostic correlation between IHC and DIF-IIF was almost 98% in most cases. IHC revealed evidence of autofluorescence around dermal blood vessels, dermal eccrine glands and neurovascular packages feeding skin appendices in ABDs; this autofluorescence may represent a non-specific immune response. Strong patterns of positivity were seen also in endothelial-mesenchymal cell junction-like structures, as well as between dermal fibrohistiocytic cells. In PV, we noted strong reactivity to neurovascular packages supplying sebaceous glands, as well as apocrine glands with edematous changes. Conclusions: We suggest that IHC is as reliable as DIF or IIF for the diagnosis of ABDs; our findings further suggest that what has previously been considered DIF/IIF autofluorescence

  12. Fluorescence In Situ Hybridization for MDM2 Amplification as a Routine Ancillary Diagnostic Tool for Suspected Well-Differentiated and Dedifferentiated Liposarcomas: Experience at a Tertiary Center

    Khin Thway


    Full Text Available Background. The assessment of MDM2 gene amplification by fluorescence in situ hybridization (FISH has become a routine ancillary tool for diagnosing atypical lipomatous tumor (ALT/well-differentiated liposarcoma and dedifferentiated liposarcoma (WDL/DDL in specialist sarcoma units. We describe our experience of its utility at our tertiary institute. Methods. All routine histology samples in which MDM2 amplification was assessed with FISH over a 2-year period were included, and FISH results were correlated with clinical and histologic findings. Results. 365 samples from 347 patients had FISH for MDM2 gene amplification. 170 were positive (i.e., showed MDM2 gene amplification, 192 were negative, and 3 were technically unsatisfactory. There were 122 histologically benign cases showing a histology:FISH concordance rate of 92.6%, 142 WDL/DDL (concordance 96.5%, and 34 cases histologically equivocal for WDL (concordance 50%. Of 64 spindle cell/pleomorphic neoplasms (in which DDL was a differential diagnosis, 21.9% showed MDM2 amplification. Of the cases with discrepant histology and FISH, all but 3 had diagnoses amended following FISH results. For discrepancies of benign histology but positive FISH, lesions were on average larger, more frequently in “classical” (intra-abdominal or inguinal sites for WDL/DDL and more frequently core biopsies. Discrepancies of malignant histology but negative FISH were smaller, less frequently in “classical” sites but again more frequently core biopsies. Conclusions. FISH has a high correlation rate with histology for cases with firm histologic diagnoses of lipoma or WDL/DDL. It is a useful ancillary diagnostic tool in histologically equivocal cases, particularly in WDL lacking significant histologic atypia or DDL without corresponding WDL component, especially in larger tumors, those from intra-abdominal or inguinal sites or core biopsies. There is a significant group of well-differentiated adipocytic neoplasms

  13. Neurofibroma involving obturator nerve mimicking an adnexal mass: a rare case report and PRISMA-driven systematic review.

    Chao, Wei-Ting; Liu, Chia-Hao; Chen, Yi-Jen; Wu, Hua-Hsi; Chuang, Chi-Mu; Wang, Peng-Hui


    Pelvic masses are a common gynecologic problem, and majority of them are diagnosed as ovarian tumors finally. Sometimes, it is hard to distinguish the origin of these pelvic masses. The following case is a solitary neurofibroma arising from the right-side obturator nerve, which was impressed as a right-side ovarian tumor initially. We reported this case, and also performed a PRISMA-driven systematic review to summary the similar cases in the literature. This review includes image, molecular and pathological findings and outcome of neurofibroma. A 33-year-old woman with a regular menstrual period denied any symptoms or signs. During her physical check-up, image examination revealed a right-side heterogeneous pelvic mass; it was suggestive of a complex of right-side ovarian tumor. A provisional diagnosis of retroperitoneal pelvic mass, probably a benign ovarian tumor, was made. Excision of the right-side pelvic mass was performed. We sent the specimens for frozen pathology, which indicated neurofibroma and lipomatous tumor and that the possibility of liposarcoma cannot be excluded. A segment of the obturator nerve was attached to the tumor and was severed. A right-side obturator nerve tear during tumor excision was observed, and a neurosurgeon was consulted for obturator nerve grafting and repair. The patient complained of mild weakness and paresthesia affecting the right leg, and we consulted a rehabilitation doctor for neuron injury. The patient's recovery was uneventful, and she was discharged eight days after the drain was removed. Further rehabilitation treatment was arranged. A neurofibroma is an uncommon pelvic retroperitoneal tumor, and it can be misdiagnosed as an adnexal mass. To our knowledge, this is a rare case of a solitary neurofibroma arising from the obturator nerve. It usually does not have any neurological deficit. We present this case to demonstrate that pelvic neurofibroma can be mistaken for an adnexal mass. This fact should be borne in mind

  14. A case report: a heterozygous deletion (2791_2805 del) in exon 18 of the filamin C gene causing filamin C-related myofibrillar myopathies in a Chinese family.

    Miao, Jing; Su, Fei-Fei; Liu, Xue-Mei; Wei, Xiao-Jing; Yuan, Yun; Yu, Xue-Fan


    Filamin C-related myofibrillar myopathies (MFM) are progressive skeletal myopathies with an autosomal dominant inheritance pattern. The conditions are caused by mutations of the filamin C gene (FLNC) located in the chromosome 7q32-q35 region. Genetic variations in the FLNC gene result in various clinical phenotypes. We describe a 43-year-old woman who suffered filamin C-related MFM, with symptoms first presenting in the proximal muscles of the lower limbs and eventually spreading to the upper limbs and distal muscles. The patient's serum level of creatine kinase was mildly increased. Mildy myopathic changes in the electromyographic exam and moderate lipomatous alterations in lower limb MRI were found. Histopathological examination revealed increased muscle fiber size variability, disturbances in oxidative enzyme activity, and the presence of abnormal protein aggregates and vacuoles in some muscle fibers. Ultrastructural analysis showed inclusions composed of thin filaments and interspersed granular densities. DNA sequencing analysis detected a novel 15-nucleotide deletion (c.2791_2805del, p.931_935del) in the FLNC gene. The patient's father, sister, brother, three paternal aunts, one paternal uncle, and the uncle's son also had slowly progressive muscle weakness, and thus, we detected an autosomal dominant inheritance pattern of the disorder. A novel heterogeneous 15-nucleotide deletion (c.2791_2805del, p.931_935del) in the Ig-like domain 7 of the FLNC gene was found to cause filamin C-related MFM. This deletion in the FLNC gene causes protein aggregation, abnormalities in muscle structure, and impairment in muscle fiber function, which leads to muscle weakness.

  15. Ultrasound and MRI findings in appendicular and truncal fat necrosis

    Robinson, Philip [Leeds Teaching Hospitals, Department of Radiology, Leeds (United Kingdom); Leeds Teaching Hospitals, Musculoskeletal Centre, X-Ray Department, Chapel Allerton Hospital, Leeds (United Kingdom); Farrant, Joanna M.; McKie, Scott [Leeds Teaching Hospitals, Department of Radiology, Leeds (United Kingdom); Bourke, Grainne [Leeds Teaching Hospitals, Department of Plastic Surgery, Leeds (United Kingdom); Merchant, William [Leeds Teaching Hospitals, Department of Pathology, Leeds (United Kingdom); Horgan, Kieran J. [Leeds Teaching Hospitals, Department of Surgery, Leeds (United Kingdom)


    The objective was to evaluate ultrasound and MRI in clinical appendicular and truncal fat necrosis. Thirty-three patients (14 men, 19 women, median age 55, range 29-95) were retrospectively evaluated. Histologically, three groups were seen: Group 1 (n = 18) consisted of patients with subcutaneous masses with septal and extrinsic oedema; in Group 2 (n = 11) necrosis occurred within lipomatous tumours and little oedema; and in Group 3 (n = 4) there were large complex masses consistent with Morel-Lavallee lesions. Two experienced radiologists reviewed MR (n = 30) and ultrasound (n = 32) images with consensus agreement. MRI was performed on a 1.5T system with T1-weighted, T2-weighted fat-suppressed and T1-weighted fat-suppressed post-intravenous gadolinium sequences obtained in two orthogonal planes. Ultrasound (linear 5- to 13.5-MHz probe) was performed in the longitudinal and short axis. Anatomical position, size, shape (oval, linear, ill-defined), internal architecture (lobules, septi or stranding), intrinsic signal characteristics, presence of surrounding pseudocapsule, extrinsic linear stranding and vascularity (gadolinium enhancement or power Doppler) were recorded. Anatomical locations were buttock/thigh (n = 17), leg (n = 6), upper limb (n = 5) and thoracic/abdominal wall (n = 5) with the majority of lesions (30 out of 33) oval/linear in shape. On ultrasound and MRI most lesions showed internal fat lobules, intervening septi and a surrounding pseudocapsule. Fat necrosis can usually be identified as containing multiple fat lobules on ultrasound and MRI despite a varying degree of inflammatory change surrounding and within the mass. (orig.)

  16. Current classification, treatment options, and new perspectives in the management of adipocytic sarcomas

    De Vita A


    Full Text Available Alessandro De Vita,1 Laura Mercatali,1 Federica Recine,1 Federica Pieri,2 Nada Riva,1 Alberto Bongiovanni,1 Chiara Liverani,1 Chiara Spadazzi,1 Giacomo Miserocchi,1 Dino Amadori,1 Toni Ibrahim1 1Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori (IRST IRCCS, Meldola, FC, 2Pathology Unit, Morgagni-Pierantoni Hospital, Forlì, Italy Abstract: Sarcomas are a heterogeneous group of mesenchymal tumors arising from soft tissue or bone, with an uncertain etiology and difficult classification. Soft tissue sarcomas (STSs account for around 1% of all adult cancers. Till date, more than 50 histologic subtypes have been identified. Adipocyte sarcoma or liposarcoma (LPS is one of the most common STS subtypes, accounting for 15% of all sarcomas, with an incidence of 24% of all extremity STSs and 45% of all retroperitoneal STSs. The new World Health Organization classification system has divided LPS into four different subgroups: atypical lipomatous tumor/well-differentiated LPS, dedifferentiated LPS, myxoid LPS, and pleomorphic LPS. These lesions can develop at any location and exhibit different aggressive potentials reflecting their morphologic diversity and clinical behavior. Patients affected by LPS should be managed in specialized multidisciplinary cancer centers. Whereas surgical resection is the mainstay of treatment for localized disease, the benefits of adjuvant and neoadjuvant chemotherapy are still unclear. Systemic treatment, particularly chemotherapy, is still limited in metastatic disease. Despite the efforts toward a better understanding of the biology of LPS, the outcome of advanced and metastatic patients remains poor. The advent of targeted therapies may lead to an improvement of treatment options and clinical outcomes. A larger patient enrollment into translational and clinical studies will help increase the knowledge of the biological behavior of LPSs, test new drugs, and introduce new

  17. Imaging appearance of well-differentiated liposarcomas with myxoid stroma.

    Morag, Yoav; Yablon, Corrie; Brigido, Monica Kalume; Jacobson, Jon; Lucas, David


    Describe the imaging appearance of well-differentiated liposarcoma with myxoid stroma (WDLMS) and correlate with histopathology. A keyword search of the institution medical records was performed from 1 January 2000 to 30 June 2017. The histopathology slides of cases identified in this fashion were then reviewed by a pathologist. Additional cases were prospectively collected from extramural referrals and tumor boards. Diagnostic imaging studies of pathologically proven cases of WDLMS were then reviewed in consensus and correlated with pathology. Ten cases of pathologically proven WDLMS were identified (7 men, 3 women, ages 26-81). Tumor location included the retroperitoneum (n = 5), thigh (n = 4), and the shin (n = 1). Nine patients had macroscopic fat on imaging. The nonlipomatous components had a variable appearance, including septal, nodular, and lacelike patterns. Two cases included two distinct areas that were predominantly myxoid or lipomatous ("bi-morphic"). One tumor had no macroscopic fat on imaging. On CT, the nonlipomatous nodular components were hypodense/had hypodense areas. On MRI, the nodular components had intermediate/bright T2W signal. Interval nonlipomatous nodular growth was identified in 3 cases. WDLMS may present on imaging as a mass with variable morphology and amounts of nonlipomatous components. Histopathological diagnosis of WDLMS is challenging and imaging correlation may be helpful, as this tumor may have ≥50% fatty volume, may have a myxoid nodular component or bi-morphic appearance, or may be located in the retroperitoneum, features that are unusual for myxoid liposarcoma. WDLMS with a nodular component cannot be distinguished from dedifferentiated liposarcoma based on imaging alone.

  18. Well-circumscribed deep-seated lipomas of the upper extremity. A report of 13 cases.

    Elbardouni, A; Kharmaz, M; Salah Berrada, M; Mahfoud, M; Elyaacoubi, M


    The purpose of this study is to determine if giant size is of bad prognosis in deep lipomas of the upper extremity. We report a retrospective study of 13 patients with deep-seated lipomas of the upper extremity treated during the period from April 1997 to April 2008. We evaluated the clinical and radiological characteristics, treatment and evolution profile of these patients. There were 10 women and three men, with an average age of 53 years (range 30-79 years). Seven of these lipomas were in the arm, one in the shoulder, and five in the forearm. Six lipomas were intramuscular, six intermuscular (three of them being attached to bone and labelled parosteal lipoma) and one epivaginal lipoma of the flexor tendon sheath. All patients presented a progressive slow-growing mass that was associated with radial paralysis in one case and carpal tunnel syndrome in one case. Plain radiographs showed a radiolucent soft-tissue image in all cases and an associated osteochondroma in one parosteal lipoma. Computer tomography (CT) or magnetic resonance imaging (MRI) suggested the lipomatous nature and benign characteristics of these deep lipomas that were giant in all cases (mean size: 7 cm). Lipoma marginal excision was performed and histopathological examination demonstrated features consistent with a benign lipoma. There was good function and no clinical recurrence was observed after a mean follow-up of three years. Giant deep-seated lipomas of the upper extremity are uncommon and can be intermuscular or intramuscular. A painless soft-tissue mass is the most frequent chief complaint. MRI with fat suppression suggests the diagnosis and studies the extension of deep lipoma. Marginal excision is the treatment of choice and histopathology eliminates diagnosis of well-differentiated liposarcoma. Appropriate evaluation of deep lipoma is to rule out malignancy by systematically performing MRI and biopsy. In contrast to deep-seated lipomas of the lower extremity or the retroperitoneal

  19. Scope of FNAC in the diagnosis of soft tissue tumors-A study from a tertiary cancer referral center in India

    Rekhi Bharat


    Full Text Available Abstract Background Fine needle aspiration cytology (FNAC forms one of the first diagnostic tools in the evaluation of tumors. Its role in diagnosing soft tissue tumors (STT has been fairly documented, as well as debated. Present study was aimed at evaluating its scope in diagnosing 127 cases of soft tissue tumors. Methods Conventional Pap and MGG staining was available in all the cases. Immunocytochemistry (ICC was performed in 15 cases. Histopathological details were available in 115 cases. Results 50% cases were referred for a primary diagnosis, while 26.8% & 22.8% cases were evaluated for recurrent and metastatic lesions, respectively. Extremities were the commonest sites. On FNAC, 101 cases (79.5% were labeled as malignant, whereas 10 cases (7.9% were labeled as benign. The remaining 16 cases (11% were not categorized and were labeled as 'unsure/not specified'. Histopathological confirmation in 115 cases, gave a diagnostic accuracy of 98%, with a positive predictive value of 98% in malignant cases and a negative predictive value of 100% in benign cases. Two cases were false positive. Among the various cytological categories, 60 cases (47.2% were of spindle cell type, followed by 32 (25.2% of round cell type and 14 cases (11% of lipomatous type. Other 12 cases (9.4% were of pleomorphic type; 7 (5.5% cases of epithelioid type and remaining 2 cases were of myxoid type. All the round cell, pleomorphic and myxoid type of tumors were sarcomas, whereas 73.3% cases of spindle cell type were labeled as 'malignant'. Exact cytological sub typing was offered in 58 cases, with rhabdomyosarcoma (RMS as the most frequently sub typed tumor. The two false positive malignant cases were of fibromatosis and a pigmented schwannoma, on biopsy. Out of 28 metastatic lesions, lymph nodes were the commonest site for metastasis, with epithelioid tumors that formed highest percentage of metastatic cases. Conclusion FNAC is fairly specific and sensitive in STT

  20. Automated brightfield dual-color in situ hybridization for detection of mouse double minute 2 gene amplification in sarcomas.

    Zhang, Wenjun; McElhinny, Abigail; Nielsen, Alma; Wang, Maria; Miller, Melanie; Singh, Shalini; Rueger, Ruediger; Rubin, Brian P; Wang, Zhen; Tubbs, Raymond R; Nagle, Raymond B; Roche, Pat; Wu, Ping; Pestic-Dragovich, Lidija


    The human homolog of the mouse double minute 2 (MDM2) oncogene is amplified in about 20% of sarcomas. The measurement of the MDM2 amplification can aid in classification and may provide a predictive value for recently formulated therapies targeting MDM2. We have developed and validated an automated bright field dual-color in situ hybridization application to detect MDM2 gene amplification. A repeat-depleted MDM2 probe was constructed to target the MDM2 gene region at 12q15. A chromosome 12-specific probe (CHR12) was generated from a pα12H8 plasmid. The in situ hybridization assay was developed by using a dinitrophenyl-labeled MDM2 probe and a digoxigenin-labeled CHR12 probe on the Ventana Medical Systems' automated slide-staining platforms. The specificity of the MDM2 and CHR12 probes was shown on metaphase spreads and further validated against controls, including normal human tonsil and known MDM2-amplified samples. The assay performance was evaluated on a cohort of 100 formalin-fixed, paraffin-embedded specimens by using a conventional bright field microscope. Simultaneous hybridization and signal detection for MDM2 and CHR12 showed that both DNA targets were present in the same cells. One hundred soft tissue specimens were stained for MDM2 and CHR12. Although 26 of 29 lipomas were nonamplified and eusomic, MDM2 amplification was noted in 78% of atypical lipomatous tumors or well-differentiated liposarcomas. Five of 6 dedifferentiated liposarcoma cases were amplified for MDM2. MDM2 amplification was observed in 1 of 8 osteosarcomas; 3 showed CHR12 aneusomy. MDM2 amplification was present in 1 of 4 chondrosarcomas. Nine of 10 synovial sarcomas displayed no evidence of MDM2 amplification in most tumor cells. In pleomorphic sarcoma, not otherwise specified (pleomorphic malignant fibrous histiocytoma), MDM2 was amplified in 38% of cases, whereas 92% were aneusomic for CHR12. One alveolar rhabdomyosarcoma and 2 embryonal rhabdomyosarcomas displayed low-level aneusomy

  1. Morpho-functional assessment of interatrial septum: a transesophageal echocardiographic study.

    Galzerano, D; Tuccillo, B; Lama, D; Paolisso, G; Mirra, G; Giasi, M


    Despite the increasing number of reports on lipomatous hypertrophy of interatrial septum, a standardization of measurement of the dimensions of the interatrial septum (IAS) in the different phases of cardiac cycle has not been reported. Moreover, no data on modification of thickness with age and in specific cardiac diseases are available. Our purpose was to study whether the changes of thickness and thinning of IAS may be related to age, left atrial dimension, cardiac cycle and different cardiac diseases. 248 patients (mean age 52.7 +/- 19.9 years) underwent transthoracic (TTE) and transesophageal (TEE) echocardiography. IAS was measured at the constant regions anterior and posterior to the fossa ovalis. IAS thickness (tk), thinning (th) and % thinning (% th) were measured. IAS thickness ranged from 4 to 13 mm at the time of ventricular end-systolic phase (mean 6.7 +/- 1.9 mm) and from 6 to 16 mm at the time of atrial systole (mean 9.9 +/- 1.8 mm); significant statistical difference between these values was found (P IAS thinning ranged from 1 to 7 mm (mean 3.42 +/- 1.8) while % IAS thinning from 18 to 76% (mean 36.53 +/- 16.36%). Statistical analysis showed a significant positive correlation between age and ventricular end-systolic thickness and atrial systolic thickness and thinning. An insignificant correlation was found between age and % IAS thinning and between left atrial dimension and IAS tk and th. Our results demonstrate that IAS thickness increases by age; no correlation exists between IAS thinning and age. There is no difference between IAS thickness and thinning in patients with or without cardiac disease. We believe that the thickness of IAS can be considered hypertrophic only if it exceeds the value of 15 mm during both ventricular end-systolic and atrial systolic phases of the cardiac cycle. IAS thickness and thinning might be an additional parameter to evaluate systolic atrial function particularly with regard to maintenance of synus rhythm after

  2. Imperfuração anal associada à agenesia parcial do sacro e lipoma pré-sacral: síndrome de Currarino Imperforate anus associated with partial sacral agenesis and presacral lipoma: Currarino syndrome

    Paulo Ricardo G. Zen


    anus and recto-vestibular fistula diagnosed in the first day after birth. At seven months of age, she started to present episodes of recurrent urinary infections and received a diagnosis of neurogenic bladder. At the same time, partial sacral agenesis was noted. Magnetic resonance imaging and computed tomography scan of the spine identified the presence of a fistula coincident with the lombo-sacral dimple described at clinical examination, amputation of the lower portion of the spinal cord with reduced number of nervous roots of the caudus equinus and lipomatous presacral mass. The patient did not present other dysmorphia. Parental radiologic evaluation did not identify sacral abnormalities. COMMENTS: Currarino syndrome is a rare autosomal dominant genetic disease characterized by the triad composed of anal atresia, partial sacral agenesis and presacral tumor. It includes, among others, teratomas, meningoceles, enteric cysts and lipomas, as observed in our patient. Children presenting anorectal abnormalities should be evaluated regarding the presence of Currarino syndrome. The partial sacral agenesis is a major sign of this disease.

  3. In vivo verification of proton beam path by using post-treatment PET/CT imaging.

    Hsi, Wen C; Indelicato, Daniel J; Vargas, Carlos; Duvvuri, Srividya; Li, Zuofeng; Palta, Jatinder


    , also show large misalignment between the field edge and the positron emission distribution in lipomatous tissues around the prostate. These motion-after-Tx cases correspond to patients with large changes in volume of rectal gas between the post-Tx and the post-PET CTs. The standard deviations for D(AP) and D(SI) are 5.0 and 3.0 mm, respectively, for these motion-after-Tx cases. The final seven studies, referred to as position-error cases, which had a large discordance but no misalignment, were found to have deviations of 4.6 and 3.6 mm in D(AP) and D(SI), respectively. The position-error cases correspond to a large discrepancy on the relative location between the centroid and pelvic bones seen in post-Tx CT and recorded x-ray radiographs. Systematic analyses of proton-activated positron emission distributions provide patient-specific information on prostate motion (sigmaM) and patient position variability (sigmap) during daily proton beam delivery. The less than 2 mm of displacement variations in the good cases indicates that population-based values of sigmap and sigmaM, used in margin algorithms for treatment planning at the authors' institution are valid for the majority of cases. However, a small fraction of PET/CT studies (approximately 14%) with -4 mm displacement variations may require different margins. Such data are useful in establishing patient-specific planning target volume margins.

  4. In vivo verification of proton beam path by using post-treatment PET/CT imaging

    Hsi, Wen C.; Indelicato, Daniel J.; Vargas, Carlos; Duvvuri, Srividya; Li Zuofeng; Palta, Jatinder [Proton Therapy Institute, University of Florida, Jacksonville, Florida 32206 (United States); Boca Radiation Oncology Associates, Boca Raton, Florida 33431 (United States); Proton Therapy Institute, University of Florida, Jacksonville, Florida 32206 (United States); Department of Radiation Oncology, University of Florida, Gainesville, Florida 32610 (United States)


    studies, referred as motion-after-Tx cases, also show large misalignment between the field edge and the positron emission distribution in lipomatous tissues around the prostate. These motion-after-Tx cases correspond to patients with large changes in volume of rectal gas between the post-Tx and the post-PET CTs. The standard deviations for D{sub AP} and D{sub SI} are 5.0 and 3.0 mm, respectively, for these motion-after-Tx cases. The final seven studies, referred to as position-error cases, which had a large discordance but no misalignment, were found to have deviations of 4.6 and 3.6 mm in D{sub AP} and D{sub SI}, respectively. The position-error cases correspond to a large discrepancy on the relative location between the centroid and pelvic bones seen in post-Tx CT and recorded x-ray radiographs. Conclusions: Systematic analyses of proton-activated positron emission distributions provide patient-specific information on prostate motion ({sigma}{sub M}) and patient position variability ({Sigma}{sub p}) during daily proton beam delivery. The less than 2 mm of displacement variations in the good cases indicates that population-based values of {Sigma}{sub p} and {sigma}{sub M} used in margin algorithms for treatment planning at the authors' institution are valid for the majority of cases. However, a small fraction of PET/CT studies (approximately 14%) with {approx}4 mm displacement variations may require different margins. Such data are useful in establishing patient-specific planning target volume margins.