Myxoid Tumor Research Articles

Axitinib Has Antiangiogenic and Antitumorigenic Activity in Myxoid Liposarcoma.

Myxoid liposarcoma is a rare form of soft-tissue sarcoma. Although most patients initially respond well to treatment, approximately 21% relapse, highlighting the need for alternative treatments. To identify novel treatment regimens and gain a better understanding of myxoid liposarcoma tumor biology, we screened various candidate and approved targeted therapeutics and chemotherapeutics against myxoid liposarcoma cell lines. Therapeutics that target angiogenesis showed antitumor activity. The small molecule inhibitor axitinib, which targets angiogenesis by inhibiting the VEGFR and PDGFR families and c-Kit, inhibited cell cycle progression and induced apoptosis in vitro, as well as having significant antitumor activity against MLS 1765 myxoid liposarcoma xenografts in mice. Axitinib also displayed synergistic antitumor activity in vitro when combined with the potassium channel ionophore salinomycin or the BH3 mimetic ABT-737. Another angiogenesis-targeting therapeutic, 4EGI-1, which targets the oncoprotein eIF4E, significantly decreased angiogenic ligand expression by myxoid liposarcoma cells and reduced tumor cell growth. To verify this oncogenic addiction to angiogenic pathways, we utilized VEGFR-derived ligand traps and found that autocrine VEGFR signaling was crucial to myxoid liposarcoma cell survival. Overall, these findings suggest that autocrine angiogenic signaling through the VEGFR family is critical to myxoid liposarcoma cell survival and that further study of axitinib as a potential anticancer therapy is warranted.

Diagnostic value of CT and MRI in myxoid soft tissue tumours

Objective To study the CT and MRI characteristics of myxoid soft tissue tumours. Methods Fifty-three myxoid soft tissue tumours pathologically confirmed were retrospectively studied. MR and CT scan were performed before operation. Noncontrast T1 and T2 weighted images, fat-suppressed T2 weighted images were obtained before contrast-enhanced scan with fat-suppressed T1 weighted sequence was underwent. Plain and contrast-enhanced CT was performed with tube potential of 120 kV, matrix 512×512, and automatic tube current modulation technique. Results There were 18 myxoid liposarcoma, 3 ganglioneuroma, 8 myxofibrosarcoma, 2 extraskeletal myxoid chondrosarcoma, 10 intramuscular myxoma, and 2 juxta-articular myxoma. Microscopic examination showed myxoid soft tissue tumours which were consisted of abundant myxoid stroma with tumour cells and blood vessels associated with fibrous bands. Low density in CT images, hypointensity in T1 weighted images, significantly hyperintense in T2 weighted images were their common manifestations in the area of abundance of extracellular myxiod matrix. Plain CT images demonstrated equidensite, T1 weighted images isointensity, and fat-suppressed T1 weighted images significant enhancement relatively in the areas of increased number of tumor cells. Hemorrhage, necrosis and capsule of the lesion were revealed in high grade malignant tumors. The septa can be identified in most of myxoid tumours of soft tissue. Conclusion CT and MRI can definitely show the image characteristics of myxiod soft tissue tumours, which can help make a qualitative diagnosis before operation. Key words: Soft tissue neoplasms; Tomography, X-ray computed; Magnetic resonance imaging

Relationship between diffusion parameters derived from intravoxel incoherent motion MRI and perfusion measured by dynamic contrast-enhanced MRI of soft tissue tumors.

Our aim was to evaluate the link between diffusion parameters measured by intravoxel incoherent motion (IVIM) diffusion-weighted imaging (DWI) and the perfusion metrics obtained with dynamic contrast-enhanced (DCE) MRI in soft tissue tumors (STTs). Twenty-eight patients affected by histopathologically confirmed STT were included in a prospective study. All patients underwent both DCE MRI and IVIM DWI. The perfusion fraction f, diffusion coefficient D and perfusion-related diffusion coefficient D* were estimated using a bi-exponential function to fit the DWI data. DCE MRI was acquired with a temporal resolution of 3-5 s. Maps of the initial area under the gadolinium concentration curve (IAUGC), time to peak (TTP) and maximum slope of increase (MSI) were derived using commercial software. The relationships between the DCE MRI and IVIM DWI measurements were assessed by Spearman's test. To exclude false positive results under multiple testing, the false discovery rate (FDR) procedure was applied. The Mann-Whitney test was used to evaluate the differences between all variables in patients with non-myxoid and myxoid STT. No significant relationship was found between IVIM parameters and any DCE MRI parameters. Higher f and D*f values were found in non-myxoid tumors compared with myxoid tumors (p = 0.004 and p = 0.003, respectively). MSI was significantly higher in non-myxoid tumors than in myxoid tumors (p = 0.029). From the visual assessments of single clinical cases, both f and D*f maps were in satisfactory agreement with DCE maps in the extreme cases of an avascular mass and a highly vascularized mass, whereas, for tumors with slight vascularity or with a highly heterogeneous perfusion pattern, this association was not straightforward. Although IVIM DWI was demonstrated to be feasible in STT, our data did not support evident relationships between perfusion-related IVIM parameters and perfusion measured by DCE MRI.

Undifferentiated myxoid lipoblastoma with PLAG1–HAS2 fusion in an infant; morphologically mimicking primitive myxoid mesenchymal tumor of infancy (PMMTI)—diagnostic importance of cytogenetic and molecular testing and literature review

Lipoblastoma is a benign myxoid neoplasm arising in young children that typically demonstrates adipose differentiation. It is often morphologically indistinguishable from primitive myxoid mesenchymal tumor of infancy (PMMTI), which is characterized by a well-circumscribed myxoid mass with a proliferation of primitive mesenchymal cells with mild cytologic atypia. PMMTI occurs in the first year of life and is known to have locally aggressive behavior. No specific genetic rearrangements have been reported to date. In contrast, the presence of PLAG1 (Pleomorphic Adenoma Gene 1) rearrangement is diagnostic for lipoblastoma. We hereby demonstrate the combined application of multiple approaches to tackle the diagnostic challenges of a rapidly growing neck tumor in a 3-month-old female. An incisional tumor biopsy had features of an undifferentiated, myxoid mesenchymal neoplasm mimicking PMMTI. However, tumor cells showed diffuse nuclear expression by immunohistochemical (IHC) stain. Conventional cytogenetic and fluorescence in situ hybridization (FISH) analyses as well as next generation sequencing (NGS) demonstrated evidence of PLAG1 rearrangement, confirming the diagnosis of lipoblastoma. This experience warrants that undifferentiated myxoid lipoblastoma can mimic PMMTI, and the combination of cytogenetic and molecular approaches is essential to distinguish these two myxoid neoplasms. Literature on lipoblastomas with relevant molecular and cytogenetic findings is summarized. Our case is the first lipoblastoma diagnosed with a PLAG1 fusion defined by NGS technology.

Abstract 5269: Myxofibrosarcoma: A move toward Precision Medicine

Abstract Background Soft tissue sarcomas are rare malignant tumors that account for approximately 1% of all malignancies. Myxofibrosarcoma (MFS) is one of the most common sarcomas in elderly patients. Because of their highly complex Karyotypes with extensive intratumoral heterogeneity, no specific recurrent aberration has emerged. In our Precision Cancer Medicine Program, we enroll patients with rare malignancies, metastatic or treatment-resistant disease in order to analyze the individual patient's disease and identify druggable targets. We sequenced two macroscopically different appearing tumor areas from a patient with a primary high-grade MFS using a Whole Exome Sequencing (WES) clinical test called EXaCT-1. Design The patient was enrolled prospectively through our Precision Medicine Clinic under an IRB approved protocol. Genomic DNA from fresh OCT (TissueTek) - frozen tissue from a myxoid hypocellular tumor area, from a more pleomorphic hypercellular tumor area and from benign tissue were extracted using the Promega Maxwell 16 MDx. Sequencing was performed using Illumina HiSeq 2500 (2×100bp). A total of 21,522 genes were analyzed using Agilent HaloPlex Exome. Reads were aligned to GRC37/hg19 reference using BWA and processed accordingly to IPM-Exome-pipeline v0.9. Sequencing results were confirmed and further analyzed using CLONET (CLONality Estimate in Tumors) and fluorescence in situ hybridization (FISH). CDK4/6 inhibitors (PD0332991, LY2835219) were tested in a MFS monolayer cell line (OH931) and an engineered 3D mini-MFS system. Results Whole exome sequencing and CLONET analysis revealed a hemizygous deletion in the myxoid hypocellular tumor area and a homozygous deletion in the pleomorphic hypercellular tumor area of the tumor suppressor cyclin-dependent kinase inhibitor CDKN2A (p16/Ink4a). Looking through the genomic data published by Barretina et al. (Nat Genet. 2010), we recognized a homozygous deletion of CDKN2A in 15% of the MFS analyzed. Screening full sections of 30 MFS with FISH for CDKN2A deletion shows intratumoral heterogeneity with hemizygous deletion, homozygous deletion and amplification of CDKN2A. CDKN2A loss may be associated with increased sensitivity to CDK4/6 inhibitors. Treatment of CDKN2A null MFS cell line OH931 (kindly provided by Dr. Julia Bridge) with CDK4/6 inhibitors (PD0332991, LY2835219) showed decreased cell proliferation compared to a CDKN2A wild-type sarcoma cell line RD (ATCC CCL-136) in monolayer culture and in our newly developed 3D system. Conclusion Tumor suppressor cyclin-dependent kinase inhibitor CDKN2A (p16/Ink4a) deletion or methylation has been reported in multiple cancer types and is correlated with worse patient outcome. Patients suffering from a CDKN2A (p16/Ink4a) null high-grade MFS may benefit from CDK4/6 inhibition as shown in a monolayer and 3D cell culture model of a MFS cell line. This study also provides proof of principle for the benefit enrolling sarcoma patients in Precision Cancer Medicine Programs. Citation Format: Chantal Pauli, Jonathan Pauwels, Theresa Y. MacDonald, Juan Miguel Mosquera, Andrea Sboner, Olivier Elemento, Francesca Demichelis, Davide Prandi, David Rickman, Beata Bode, Himisha Beltran, Mark A. Rubin. Myxofibrosarcoma: A move toward Precision Medicine. [abstract]. In: Proceedings of the 106th Annual Meeting of the American Association for Cancer Research; 2015 Apr 18-22; Philadelphia, PA. Philadelphia (PA): AACR; Cancer Res 2015;75(15 Suppl):Abstract nr 5269. doi:10.1158/1538-7445.AM2015-5269

Primary renal myxofibrosarcoma

Kidney sarcomas are rare, representing only 1% of malignant renal tumors. We herein report the case of a 70-year-old woman that was admitted for an episode of confusion in relation to hypertensive encephalopathy. Imaging investigations revealed a large mass in the right kidney with extension to the renal hilum. The patient underwent right open radical nephrectomy. The histopathologic study disclosed a 15-cm, myxoid and cellular, pleomorphic tumor with elongated, curvilinear, thin-walled vessels, and focal necrosis that involved the upper and middle segments of the kidney. Immunohistochemically, the tumor cells showed strong positivity for vimentin, bcl2 protein (nuclear staining pattern), CD34, CD99, and alpha-methylacyl coenzyme A racemase. The tumor was diagnosed as myxofibrosarcoma (MFS) grade 2 according to the FNCLCC system. To the best of our knowledge, this is the first report of an MFS arising from the kidney. Thus, MFS is an uncommon soft tissue tumor that can exceptionally arise from the kidney. The differential diagnosis with other myxoid tumors is of vital importance because it includes lesions with subtle differences and extremely variable biological behavior. Radical surgery is the treatment of choice. Long-term follow-up is recommended because of the tumor's capability for local recurrence and distant metastasis.

Intense Lubricin Expression Defines Synoviocyte Differentiation and Detects Cells in Synovium‐Related and Myxoid Tumors

Immunoprofiles of synovium-related tumors, such as Giant Cell Tumor of Tendon Sheath (GCTTS), are incompletely explored, partly due to lack of lineage defining synoviocyte markers. Synovial lining is comprised of intimal macrophages and fibroblast-like synoviocytes (FLS). FLS produce lubricin, a mucin-like glycoprotein in synovial fluid. Lubricin expression in synovium, GCTTS and myxomas was assessed by immunohistochemistry using two anti-lubricin antibodies: S6.79, monoclonal antibody, raised against purified, native lubricin, and Ab28484, an affinity purified polyclonal antibody made against a C-terminal peptide. In synovium, lubricin reactivity was limited to intimal synoviocytes with cytoplasmic and surface lining staining Double staining using Ab28484 and anti-CD163, a marker of synovial intimal macrophages, showed non-overlap between the two synoviocyte types. In GCTTS, lubricin cytoplasmic expression was limited to large, plump cells, but not giant cells or smaller mononuclear cells Double staining showed lubricin positive cells were CD163 negative. Scattered mononuclear cells negative for both lubricin and CD163were present. A case of juxta-articular myxoma with extensive ganglion cyst-like areas showed moderate staining of myxoid stroma and intense staining of cyst lining using S6.79. A tumor of uncertain lineage showed reactivity, suggesting relatedness to FLS. Thus, lubricin expression defines cells within synovium-related and myxoid tumors.

Transformation of a Primitive Myxoid Mesenchymal Tumor of Infancy to an Undifferentiated Sarcoma

An 8-month-old girl underwent surgical resection of a cervical mass with histologic diagnosis of a primitive myxoid mesenchymal tumor of infancy (PMMTI). More than 5 years after the initial surgical intervention, the tumor recurred locally, with numerous distant metastases. The histologic morphology of this tumor was compatible with a diagnosis of an undifferentiated high-grade sarcoma. PMMTI is a recently described poorly differentiated fibroblastic soft-tissue tumor of infancy, of at least borderline biological behavior, characterized by local recurrence and a potential to metastasize. We present here the first case of a transformation of a PMMTI into an undifferentiated high-grade sarcoma.

Myxoid soft-tissue neoplasms: comprehensive update of the taxonomy and MRI features.

OBJECTIVE. The purpose of this article is to review the classification, clinical presentation, and histopathologic and MRI features of myxoid soft-tissue neoplasms. CONCLUSION. MRI is the modality of choice for characterization of myxoid soft-tissue tumors. A combination of imaging features (including certain characteristic signs), clinical features, and patient demographics can help the radiologist in coming to a specific diagnosis or in narrowing down the differential diagnoses.

Diagnostic Pitfalls of Differentiating Cellular Digital Fibroma from Superficial Acral Fibromyxoma.

Dear Editor: Acquired digital fibrokeratoma is an exophytic tumor with hyperkeratotic epidermis on acral sites. Meanwhile, cellular digital fibroma (CDF) is a unique subset of acquired digital fibrokeratomas that comprise slender spindle-shaped CD34-positive cells1. Here, we report a rare case of CDF and its clinicopathologic characteristics. A 38-year-old Korean man presented with a painless nodule, which began to grow six months prior to examination, on the ventral part of the proximal phalanx of his right index finger. Physical examination revealed a fixed erythematous protruding nodule 0.5 cm in diameter (Fig. 1A). The results of punch biopsy for complete removal showed a polypoid tumor constricted at its base and lateral sides by acanthotic epidermis with collarette-like changes (Fig. 1B). Collagenous bundles were filled mostly with myxoid materials and numerous spindle cells arranged in a loose fascicular pattern (Fig. 1C). No nuclear atypia or mitoses were identified. Immunohistochemistry revealed most proliferative spindle cells were positive for CD34 (Fig. 1D) and vimentin (Fig. 1E) but negative for CD99, S-100, and smooth muscle actin. Prominent myxoid stroma was detected by Alcian blue stain (Fig. 1F). On the basis of these findings, the lesion was diagnosed as CDF. After the lesion was completely removed, the patient did not experience any recurrence over 18 months of follow-up. Fig. 1 (A) An erythematous fixed protruding nodule 0.5 cm in diameter on the ventral part of the proximal phalanx of the patient's right index finger. (B~F) Low-magnification view of the specimen indicates a polypoid tumor protruding above the surrounding skin. ... Since McNiff et al.1 first described CDF in 2005, their observations have been regarded as important for the diagnosis of fibrohistiocytic neoplasms, which can be easily misdiagnosed as superficial acral fibromyxoma (SAFM)2. We initially considered a diagnosis of SAFM in our case; however, clinically, SAFMs are slow-growing asymptomatic tumors that commonly present as solitary lesions ranging from 0.5 to 5 cm in diameter3,4. The lesion in the present case was relatively smaller than typical SAFMs. Histopathologically, both SAFM and CDF can be characterized by spindle to stellate cells arranged in a storiform or fascicular pattern with variable degrees of myxoid background stroma5. Accentuated microvasculature and conspicuous mast cells are potentially suggestive of SAFM; meanwhile, keratin horn and epidermal collarette are commonly observed in CDF4,5. In our case, the nodule showed proliferation of spindle cells in a loose fascicular pattern; however, it was constricted at its base and lateral sides by collarette-like acanthotic epidermis. No vascular proliferation, atypical mitoses, or fibroblasts oriented vertically to the axis of the lesion were observed. In addition, the lesion was negative for CD99. Therefore, the tumor was consistent with a diagnosis of CDF. If lesions resemble myxoid fibrohistiocytic tumors (Table 1), immunohistochemistry can be used for an accurate diagnosis of CDF. Table 1 Characteristics of myxoid soft-tissue tumors mimicking cellular digital fibroma McNiff et al.1 report that the natural course of CDF appears to be benign owing it its small size, unremarkable cytology, and absence of recurrence after biopsy. However, elucidating the detailed pathophysiology and clinical characteristics of CDF requires additional case reports. Rare cases of CDF, such as the one presented herein, must be distinguished from other cutaneous fibrohistiocytic tumors including SAFM.

Useful MRI Features for Distinguishing Benign Peripheral Nerve Sheath Tumors and Myxoid Tumors in the Musculoskeletal System

There are many types of relatively common soft tissue tumors, including myxoid tumors and benign peripheral nerve sheath tumors (BPNSTs). BPNSTs constitute more than 10% of all benign tumors, while myxoid tumors constitute about 3.2% of all benign tumors and over 11% of all malignant soft tissue tumors (1-5). Myxoid This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Received: July 13, 2015 Revised: September 7, 2015 Accepted: September 11, 2015

Aggressive angiomyxoma presenting as a huge intra-abdominal mass in a pregnant Nigerian woman: a case report

Aggressive angiomyxoma is a rare, locally aggressive myxoid mesenchymal tumour that predominantly arises from the pelvis and perineum of young adult females. Occasional cases have been reported at unusual sites. Oestrogen and progesterone may play a role in the development of the tumour especially during pregnancy. A 25 year old Nigerian woman presented with severe abdominal pain shortly after a fresh still birth. Clinical and radiological evaluation revealed a massive intra-abdominal mass which was excised and pathologically diagnosed as aggressive angiomyxoma. The follow up period has been uneventful. This case demonstrates that aggressive angiomyxoma may present as a primary intra-abdominal mass and thus should be considered in the differential diagnosis of such lesions as this will greatly impact on its surgical management. Moreover, pregnancy appears to influence rapid tumour growth and may result in massive tumour sizes. Long term patient follow-up is routinely advocated for the early detection of tumour recurrence.

Aggressive angiomyxoma of the vulva: a bizarre perineal lesion.

Introduction. Aggressive angiomyxoma is a rare, slowly growing, and benign tumour of mesenchymal origin, which affects women of reproductive age and is associated with a high risk of local recurrence. Case Presentation. A case of a 47-year-old white female is presented herein, with a large polypoid, gelatinous mass on the right labia majora, measuring 26 × 21 × 6 cm. Histopathologically, the lesion was composed of spindle and stellate-shaped cells embedded in a myxoid matrix. Another specific feature was the presence of variable-sized thin-walled capillaries and thick-walled vascular channels. The patient underwent wide local excision of the tumour with clear margins and developed local recurrence 18 months later. Discussion. Aggressive angiomyxoma of the vulva needs to be distinguished from benign myxoid tumors with a low risk of local recurrence as well as from malignant myxoid neoplasms. Usually wide local excision with tumour-free margins and occasionally hormonal manipulation is the treatment of choice.

Les tumeurs myxoïdes des tissus mous profonds

Myxoid soft tissue tumors form a heterogeneous group. Their biological potential encompasses the whole spectrum from benign to highly malignant. The present article focuses on myxoid tumors of the deep soft tissues: myxofibrosarcoma, low-grade fibromyxoid sarcoma, myxoma, myxoid liposarcoma, extraskeletal myxoid chondrosarcoma and nodular fasciitis. The last two decades have brought into practice multiple powerful tools that support pathologists in making precise diagnoses, even on small incisional biopsies: detection of fusion transcripts by rt-PCR, detection of chromosomal fusion or breakpoint by FISH, detection of point mutations by PCR and expression of specific markers by immunohistochemistry. Conventional morphology remains the mainstay of diagnosis, and it is essential to obtain adequate clinical and radiological information before interpreting small incisional biopsies. The present article is a summary of morphologic features used to diagnose the most common tumors of the deep soft tissues.

Primary subcutaneous myxoid liposarcoma: a clinicopathologic review of three cases with molecular confirmation and discussion of the differential diagnosis.

Myxoid liposarcoma typically presents as a deep-seated mass in the lower extremity of adults. Presentation as a primary subcutaneous tumor is rare. Here we discuss clinicopathologic characteristics of three such cases and their differential diagnosis to alert dermatopathologists to this unusual clinical presentation of a potentially aggressive entity. Cases of myxoid liposarcoma were retrieved from archives and consultation files. Inclusion required location above the subcutaneous fascia with no evidence of a metastatic origin. Clinicopathologic features were retrospectively reviewed. Fluorescence in situ hybridization for DDIT3 (CHOP) gene rearrangement was performed on all cases. The tumors affected young adults (two males and one female, mean 36 years, range 32-40 years). No prior history of myxoid liposarcoma or deep soft tissue mass was identified. The tumors occurred in the foot, thigh and hand. All demonstrated multilobular architecture with abundant myxoid stroma, prominent branching capillary vascular network and lipoblastic differentiation. No dermal involvement was seen. Round cell features were identified in one case and represented <5% of the tumor. All patients remain disease-free following local excision only at 6, 8 and 13 months. Myxoid liposarcoma can rarely present as a primary subcutaneous mass and should be considered in the differential diagnosis of cutaneous myxoid tumors in adults.

Extraskeletal Myxoid Chondrosarcoma Presenting as an Intradural Spinal Mass: Report of a Rare Clinical Presentation with an Emphasis on Differential Diagnostic Considerations

Extraskeletal myxoid chondrosarcoma is a rare soft tissue neoplasm that occurs predominantly in the soft tissues of the lower extremities. Herein we present a case of a 29 year old male who presented with bilateral femoral numbness believed to be the result of prior injury to his back. A magnetic resonance imaging revealed a mass in the T4-T5 epidural space compressing the spinal cord. Laminectomy was performed and the lesion removed piecemeal. The pathology specimen consisted of multiple fragments of dura involved by a myxoid neoplasm with a nodular growth pattern. The tumor cells were arranged in anastomosing cords and strands. Individual tumor cells were small, of uniform size and shape, with small hyperchromatic nuclei and scant eosinophilic cytoplasm. Immunohistochemical stains were performed which showed the tumor cells were diffusely positive for vimentin and focally positive for EMA, S-100 protein and cytokeratin, whereas they were negative for CD34 and CD99. Fluorescence in situ hybridization (FISH) studies showed a clonal population of cells with re-arrangement of the EWSR1 locus, confirming the histologic impression of extraskeletal myxoid chondrosarcoma. This is the first report of a case of an extraskeletal myxoid chondrosarcoma arising from the dura, confirmed to have rearrangement of the EWSR1 gene by FISH. There have only been two other cases of dural based extraskeletal myxoid chondrosarcoma reported prior to our case. We also briefly review the published literature and discuss differential diagnostic considerations for this rare tumor.

Rerouting technique of the facial nerve in the procedure of removal of jugular foramen tumors: Outcomes and indications

But de la presentation To analyze retrospectively the functional outcome of the facial nerve after different nerve rerouting techniques in the procedure of removal of jugular foramen tumors, and to discuss their indications. Materiel et methodes From 2004 to 2013, 47 patients with jugular foramen tumor were operated on, in which 40 were totally, six near-totally and 1 partially resected. The pathologies consisted of 35 jugular paragangliomas, six schwannomas, 4 chordomas, one synovial sarcoma, and one ossifying myxoid tumor. According to the location and extension of the tumors, infra-temporal fossa type A (IFTA) approach was carried out in 23 cases, infra-temporal fossa combined translabyrinthine approach in 10 cases, petro-occipital trans-sigmoid approach (POTS) in 14 cases. The total rerouting of the facial nerve was done in 14 cases, partial rerouting in 12 cases, and no rerouting in 21 cases. Resultats These patients were followed up for 1-10 years. The facial nerve function after one year was HB I-II in 28 cases, HB III-IV in 17 cases, and HB V-VI in two cases. The facial nerve function after three years was HB I-II in 35 cases and HB III-IV in eight cases. The facial nerve function was deteriorated in 18 cases, improved in two cases, and unchanged in 27 cases. Conclusion The intraoperative facial nerve injury is a common complication in the surgery of jugular foramen tumors. The nature, size, location of tumors, preoperative facial function, surgical approach and intraoperative nerve management could affect the prognosis of facial nerve function. Partial or no rerouting of the facial nerve could be used in the procedure of removal of jugular foramen tumors in order to obtain better facial function. The tumors, those posterior and inferior to the labyrinth, could be removed via IFTA with partial or no rerouting of the facial nerve or POTS.

Rerouting Technique of the Facial Nerve in the Procedure of Removal of Jugular Foramen Tumors: Outcomes and Indications

But de la presentation To analyze retrospectively the functional outcome of the facial nerve after different nerve rerouting techniques in the procedure of removal of jugular foramen tumors, and to discuss their indications. Materiel et methodes From 2004 to 2013, 47 patients with jugular foramen tumor were operated on, in which 40 were totally, six near-totally and 1 partially resected. The pathologies consisted of 35 jugular paragangliomas, six schwannomas, 4 chordomas, one synovial sarcoma, and one ossifying myxoid tumor. According to the location and extension of the tumors, infra-temporal fossa type A (IFTA) approach was carried out in 23 cases, infra-temporal fossa combined translabyrinthine approach in 10 cases, petro-occipital trans-sigmoid approach (POTS) in 14 cases. The total rerouting of the facial nerve was done in 14 cases, partial rerouting in 12 cases, and no rerouting in 21 cases. Resultats These patients were followed up for 1-10 years. The facial nerve function after one year was HB I-II in 28 cases, HB III-IV in 17 cases, and HB V-VI in two cases. The facial nerve function after three years was HB I-II in 35 cases and HB III-IV in eight cases. The facial nerve function was deteriorated in 18 cases, improved in two cases, and unchanged in 27 cases. Conclusion The intraoperative facial nerve injury is a common complication in the surgery of jugular foramen tumors. The nature, size, location of tumors, preoperative facial function, surgical approach and intraoperative nerve management could affect the prognosis of facial nerve function. Partial or no rerouting of the facial nerve could be used in the procedure of removal of jugular foramen tumors in order to obtain better facial function. The tumors, those posterior and inferior to the labyrinth, could be removed via IFTA with partial or no rerouting of the facial nerve or POTS.

Myxoid Variant of So-called Angiomatoid “Malignant Fibrous Histiocytoma”

Angiomatoid "malignant fibrous histiocytoma" (AMFH) is a tumor of intermediate malignancy and undefined lineage, mostly arising in the extremities of young patients. Examples with a prominent myxoid matrix are very uncommon. Twenty-one cases of myxoid AMFH (among a total of 414) identified in consult files are described, including clinicopathologic features, fluorescence in situ hybridization analysis in a subset of cases, and follow-up. Thirteen patients were female and 8 male, ranging in age from 2 to 51 y (median 17 y). These circumscribed tumors arose in subcutaneous or deep somatic soft tissue, with a median size of 2.5 cm (range, 1 to 8 cm), being located in the extremities (14/21), trunk (4/21), and upper limb girdle (3/21). Characteristic features included a fibrous pseudocapsule (20/21), peritumoral lymphoplasmacytic infiltrates (20/21), blood-filled cystic spaces (17/21), and prominent myxoid morphology comprising 60% to 100% of the tumor surface area examined. Histiocytoid or spindled tumor cells exhibited vesicular nuclei, inconspicuous nucleoli, palely eosinophilic cytoplasm, and multinodular growth without necrosis. Mucin pools and scattered multinucleate giant cells were observed in a subset of cases. Mild to moderate atypia was observed in 4 cases; 1 tumor showed a pseudochondroid matrix. Immunohistochemically, 14/21 cases expressed desmin, 12/21 expressed EMA, and 4/7 exhibited EWSR1 rearrangement. Follow-up, available for 11 patients (median, 43 mo), revealed that 3 developed local recurrence after 2, 7, and 48 months, respectively. All patients were alive without metastases. AMFH may present with prominent myxoid features making diagnosis difficult and causing possible confusion with other myxoid tumors including low-grade fibromyxoid sarcoma, extraskeletal myxoid chondrosarcoma, and myxoid liposarcoma.

Microdebrider Use in Orbital Surgery

Purpose: To report the novel use of a sinus microdebrider for the removal of tissue during orbital surgery.Methods: This retrospective study reviewed the logs of 3 surgeons to identify patients who required orbital surgery during which the surgeon chose to use a sinus microdebrider with an open sky technique as a means of removing portions of the orbital tissue. Collected data included patient demographics, clinical examinations, pathologic diagnoses, radiologic studies, operative reports and, when available, photographs and intra-operative video.Results: Three patients were identified as having undergone orbital surgery assisted by the use of a sinus microdebrider. The first patient had an extensive, recurrent left orbital myxoid tumor. Debulking of this gelatinous, infiltrative mass was aided by the combined suction and cutting action of the microdebrider. Two cases involved orbital exenteration for infiltrative sino-orbital fungus infection resulting in a blind eye and frozen globe. Removal of orbital apical tissue during exenteration surgery was facilitated with the microdebrider.Conclusions: The characteristics of the sinus microdebrider make it a useful adjunct for orbital surgery, particularly in situations where tissue may be difficult to grasp and excise. Caution should be exercised whenever using this electrically powered tool due to its potential for rapid tissue destruction. Therefore, the microdebrider should only be used in cases in which there is little risk of damage to essential orbital structures.