Soft Tissue Myxoma Research Articles

Sub-mandibular soft tissue myxoma in a teenager patient

Sub-mandibular soft tissue myxoma in a teenager patient

A Soft Tissue Myxoma on the Palate: A Case Report

A Soft Tissue Myxoma on the Palate: A Case Report

ORAL SOFT TISSUE MYXOMA: CASE REPORT

The soft tissue myxoma in the maxillofacial region is a rare benign neoplasm, with low recurrence, slow and painless growth. A 54-year-old male sought treatment with increased volume in anterior region of maxilla, evolving for 10 years asymptomatically, after extraction of tooth 11. Clinically, it had normal mucosal appearance, with a soft consistency, located in the canine sulcus region. A low-density image compatible with ovoid-shaped cyst was observed on the tomography in primary buccal fascial space. According to clinical and imaging examination the diagnostic hypothesis was lipoma. The lesion was treated by surgical excision. Macroscopically presented a gelatinous consistency, measuring 52 x 42 x 32 mm, spherical appearance with brown coloration. Histology showed arranged cells of stellate, fusiform, or rounded shape. In the fundamental myxoid substance small islands of odontogenic epithelial remains could be found, findings compatible with myxoma. The follow-up of 3 years showed no signal of recurrence.

”Myxoma of the Jaws”: Report of an atypical presentation and review of literature

Oral lesions of myxomatous origin are relatively rare and include nerve sheath myxomas, soft tissue myxomas, oral focal mucinosis, and odontogenic myxomas. These lesions have a common histologic appearance of abundant myxoid ground substance. A 61-year-old edentulous female presented with nasal stuffiness since the past 4 months. Clinical examination revealed an asymptomatic intraoral swelling in the edentulous maxillary ridge which had ulcerated. Plain film radiography and CT showed an expansile osteolytic lesion involving the right maxillary sinus. A partial maxillectomy was performed and direct microscopy showed a predominant embryonic mesenchymal stroma with fibroblasts and calcifications suggestive of a myxoma. The central myxoma is hypothesized to originate from the odontogenic apparatus due to its almost exclusivity to the jaw bones. However, when a central myxoma occurs in an edentulous jaw at an age when the odontogenic apparatus is apparently nonfunctional, explaining the genesis of this unique tumor based on present understanding becomes difficult. We present one such case while reviewing the existing literature on the present concepts of pathogenesis.

Mazabraud's Syndrome - A Diagnosis Commonly Missed.

Introduction:Mazabraud’s syndrome is a rare benign disorder characterized by the association of single or multiple intramuscular myxomas with monostotic or polyostotic form of fibrous dysplasia. Around 80 cases have been described till date; however, a higher incidence may be expected due to silent nature of the disease and missed diagnosis. The authors report one such case of a missed diagnosis.Case Report:A 39-year-old lady presented with recurrent soft tissue myxomas in thigh. A thorough evaluation with radiographs and magnetic resonance imaging (MRI) and a clinical suspicion of this rare entity helped in making the diagnosis of Mazabraud’s syndrome. The patient was treated with wide excision of the recurrent thigh swelling and watchful observation of two other asymptomatic swelling detected on MRI examination. Bisphosphonate therapy was given for fibrous dysplasia. At latest follow-up after 2 years, the patient was asymptomatic with no recurrence.Conclusion:The report highlights the importance of a high degree of suspicion of this clinical entity in patients treated for recurrent soft tissue swelling as both myxomas and fibrous dysplasia behave differently than their isolated counterparts.

Peripheral Odontogenic Myxoma: Report of Two New Cases with a Critical Review of the Literature

Introduction: Odontogenic myxoma is a benign mesenchymal odontogenic tumor that is more frequently found in the mandibular molar region. The extra-osseous counterpart, called Peripheral Odontogenic Myxoma (POM), is an exceedingly rare entity and with very low aggressive potential. Methods: Clinical and radiographic findings are not specific, and difficulties may exist in differentiating histological findings of POM from other tumors with myxoid features, such as fibromas and oral focal mucinosis. Due to the fact that the presence of odontogenic epithelium is not required for the diagnosis, a myxoid lesion without odontogenic nests that develops in extraosseous position can be misdiagnosed with a soft tissue myxoma. Case Reports: We thereby present two cases of POM and conduct an extensive and critical review of the English literature, taking into consideration both certain and putative cases of POM.

ODONTOGENIC MYXOMA VERSUS SOFT TISSUE MYXOMA: IMMUNOHISTOCHEMICAL STUDY

Background: Odontogenic Myxoma (OM) and Soft Tissue Myxoma (STM) are two myxoid tumors that may arise in the oral cavity. They often present a diagnostic and treatment challenge because of the histological overlap at the light microscopial level. Our study aimed at understanding their origin and biological behavior through comparing the immunohistochemical expression level of the neural stem cell marker; Nestin (N), as well as the invasion marker; Matrix Metalloprotienase 9 (MMP-9). Methods: The N and MMP-9 expression area percent, were measured and calculated in a total of 16 Myxoma specimens, where 9 cases of OM and 7 cases of STM were examined. Results: N and MMP-9 were positively expressed in both OM and STM showing maily cytoplasmic immunostaing, where higher expression level in OM was found; using both markers. Conclusions: Finally, our results were able to suggest the common origin for OM and STM to be the odontogenic ectomesenchyme which is derived from neural crest cells. We propose that STM might be considered the peripheral entity of OM. Moreover, OM is less differentiated and more aggressive than STM.

Peripheral myxoma of the infratemporal region: An unusual case report

Odontogenic myxoma (OM) is a rare locally invasive benign neoplasm, almost exclusively occurring in the jawbones, comprising 3–6% of all the odontogenic tumors. The mandible is more commonly involved than the maxilla. Intraoral soft tissue myxoma is an extremely rare lesion and only few reports are available in the literature. We present probably the first of its kind in literature a soft tissue OM occurring in an unusual location: the infratemporal fossa.

Soft tissue myxoma of the cheek

Soft tissue myxomas are extremely rare, slowly growing, locally infiltrative, benign, connective tissue tumors that occur in all age groups with a peak in the fourth decade of life. In the maxillofacial region soft tissue myxomas have been found in the skin and subcutaneous tissue, fascia, parotid glands, larynx, tonsils and ears. An intraoral location of myxoma is very rare. This article presents a case report of a rare soft tissue myxoma arising in the cheek of a 44-year-old female patient.

NY-ESO-1 is a sensitive and specific immunohistochemical marker for myxoid and round cell liposarcomas among related mesenchymal myxoid neoplasms

Myxoid and round cell liposarcomas constitute approximately one-third of all liposarcomas, a relatively common group of fat-derived soft tissue sarcomas. The histomorphology is a continuum between highly differentiated myxoid and poorly differentiated round cell components. The gold standard of diagnosis is dependent on histomorphology and/or identification of t(12;16)(q13;p11) translocation by cytogenetics or demonstration of DDIT3 rearrangements by fluorescence in situ hybridization. There are currently no diagnostic immunohistochemical stains available. The broad range of myxoid neoplasms in the differential diagnosis includes a variety of sarcomas. Given the notable differences in disease biology among myxoid neoplasms, which range from benign to aggressive, an accurate diagnosis is imperative for proper treatment and prognostication. Prompted by our recent study showing frequent expression of the cancer testis antigen NY-ESO-1 in myxoid and round cell liposarcomas, we sought to evaluate the utility of NY-ESO-1 as an immunohistochemical marker for myxoid and round cell liposarcoma among mesenchymal myxoid neoplasms within the differential diagnosis. Formalin-fixed, paraffin-embedded blocks were obtained for the following mesenchymal myxoid neoplasms (n=138): myxoid and round cell liposarcoma (n=38); well-differentiated liposarcoma (n=12); lipoma (n=20; 4 with myxoid change); extra-cardiac soft tissue myxoma (n=39); extraskeletal myxoid chondrosarcoma (n=12); myxofibrosarcoma (n=10: 5 low grade, 2 intermediate grade, 3 high grade); and low-grade fibromyxoid sarcoma (n=7). Utilizing standard immunohistochemistry protocols, full sections were stained with NY-ESO-1 (clone E978), and staining was assessed for intensity (1–2+), percentage of tumor positivity, and location. In all, 36/38 (95%) of the myxoid and round cell liposarcomas demonstrated NY-ESO-1 immunoreactivity. The majority of the positive cases (34/36; 94%) showed strong, homogenous staining (>50% tumor positivity), and two cases (6%) showed weak (1+ intensity), patchy staining (20–30% tumor positivity). Immunoreactivity was predominantly cytoplasmic. All the other neoplasms evaluated were negative for NY-ESO-1. NY-ESO-1 appears to be a sensitive and a specific marker for myxoid and round cell liposarcoma among mesenchymal myxoid neoplasms. The assessment of NY-ESO-1 expression by immunohistochemistry in the appropriate setting provides a cheaper, faster, and more accessible confirmatory test.

Presentation of Intramuscular Myxoma as an Unusual Neck Lump

Intramuscular myxoma (IM) has a distinct diagnostic identity among soft tissue myxomas. IMs have an approximate incidence of 1 per million of the population per year, with a female-to-male ratio of 14:3. The age range for presentation is 40 to 70 years, and the thigh is affected most frequently. IMs most commonly affect larger muscle groups, making the head and neck a rare site. To the authors' knowledge, there is 1 previous report of an IM presenting in the sternocleidomastoid muscle. In addition, IMs usually present as slow-growing asymptomatic swellings. Although abnormal gag reflexes have been reported in cases of glossopharyngeal schwannoma and neurofibroma in patients with neurofibromatosis-1, a gag reflex has not been reported previously as a complication of IM in the head and neck. A case of IM in the left sternocleidomastoid muscle, presenting with an intense gag reflex on palpation, in a 70-year-old woman is presented.

Spectrum of Cutaneous and Soft Tissue Lesions in Two Carney Complex Patients—Adnexal Induction Versus Authentic Adnexal Neoplasms

Two unusual Carney complex patients are described. In one of them, several cutaneous biopsies revealed myxoid lesions that were rather more close to authentic adnexal neoplasms with myxoid stroma than to a "myxoma with an epithelial component." These included lesions resembling trichofolliculoma, infundibular cyst, and trichodiscoma. Additionally, 1 soft tissue myxoma was unique in the sense that it greatly resembled a cardiac myxoma, begging the question whether this could represent an embolus from the patient's cardiac myxoma. Given the large size and complexity and heterogeneity of the cutaneous lesions, the authors suggest that these may represent authentic cutaneous neoplasms accompanied by myxoid stroma and not adnexal elements induced by the stroma. However, the latter mechanism is well recognized and demonstrated by our second patient in whom adnexal-type elements in the cutaneous lesion were clearly induced by the myxoid stroma but were unusually complex by manifesting panfollicular and also sebaceous differentiation.

Odontogenic and oral soft tissue myxomas: clinicopathologic analysis of 16 cases from Ile-Ife, Nigeria

Odontogenic myxomas (OMs) are considered slow-growing tumours with the potential for extensive bone destruction, cortical expansion, and a relatively high recurrence rate. We analysed the cases histologically diagnosed as OM in our centre and compared these to the data found in the literature.A record-based study of OMs histologically diagnosed at the Obafemi Awolowo University Teaching Hospital Complex (OAUTHC), Ile-Ife, Southwest Nigeria between 1990 and 2009 was conducted. The tumours were classified as myxomas, fibromyxomas or myxofibromas depending on the histologic picture. Gingival masses with similar histologic features but not showing bone involvement were included in the series as soft tissue myxomas.A total of 16 histologically diagnosed cases were recorded, 11(68.8%) in females and 5 (31.2%) in males giving a male:female ratio of 1:2.2. The most common tumour site was the maxilla (7 cases, 43.8%) and the mean age of the patients at the time of diagnosis was 31.1+/-18.0 years. Myxomas, exhibiting complete myxomatous tissue (8 cases, 50%) with no appreciable fibrous component were the most common histological type.The peak incidence was in the 2nd decade of life and there was a predilection for females (M:F = 1:2.2) and the maxilla. The recommended treatment of choice is radical surgery or conservative excision depending on tumour size.

Oral Soft Tissue Myxoma

The intraoral soft tissue myxoma is an extremely rare, slowly growing, benign mesenchymal tumor. The myxomas are insidious, infiltrative tumors which occurred in all age groups, with an average patient age of occurrence of 38 years. Most frequent locations were the cheek, floor of the mouth and palate. The lesions were present from 2 weeks to 6 years prior to treatment. The recommended therapy is surgical resection with adequate margins. We present a rare case report of soft tissue myxoma arising from the tongue in a 62-year-old male patient.

Pachydermoperiostosis associated with Glomerulonephritis: A Case Report

Hypertrophic osteoarthropathy (HOA) is characterized by clubbing fingers and periostosis of the long bones, which can be classified as the primary or secondary form. Pachydermoperiostosis (PDP), the primary idiopathic form of HOA, is featured by pachydermia, clubbing fi ngers, and periostosis of long bones, but lack of signs of cardiopulmonary, hepatic or intestinal diseases. Other manifestations include arthritis, seborrhea, folliculitis, cutis verticis gyrata, hypertrophic gastropathy and acroosteolysis. Here we report a 43 year-old male who had clubbing of the terminal phalanges, thickening of leg and forearm skin, progressive coarsening of facial features and seborrhea for more than a decade, came to our clinic due to both knee arthritis and pitting edema of lower limbs for about one year. The histology of skin and extremity x-ray findings were also consistent with the diagnosis of PDP. In addition, owing to heavy proteinuria, renal biopsy was performed and showed mesangial proliferative glomerulonephritis. He also had a myxoma over the right forearm. Described to date, there was no report of PDP associated with glomerulonephritis and soft tissue myxoma in the literature. Because similar pathogenic mechanisms involved in the changes of skin, bone and kidney have been revealed, we suggest that the coexistence of PDP and glomerulonephritis can be pathogenically rather than coincidentally related.

Fibrous dysplasia of bone associated with soft-tissue myxomas as well as an intra-osseous myxoma in a woman with Mazabraud's syndrome: a case report

IntroductionMazabraud's syndrome is a rare but well-described disorder characterized by fibrous dysplasia in single or multiple bones associated with one or more soft-tissue myxomas. In this report, we describe what is, to the best of our knowledge, the first case involving an intra-osseous myxoma. This finding supports, and could provide new insight into, the pathological association between fibrous dysplasia and myxomas.Case presentationIn this report, we describe the case of a 49-year-old Caucasian woman known for years to have fibrous dysplasia in the left femur and tibia who presented with progressive pain in her left leg and soft swelling in the left quadriceps region. After surgical intervention with excision of the soft-tissue mass, the diagnosis of Mazabraud's syndrome was confirmed. During follow-up, our patient presented with a painless mass located on the lateral side of the left knee, next to a second, intra-osseous lesion with the same characteristics in the left lateral tibial plateau. The histopathological examination was consistent with a soft-tissue intra-osseous myxoma.ConclusionIn the international literature, 67 cases of Mazabraud's syndrome have been described so far.To our knowledge, the present case report is the first to describe the combination of polyostotic fibrous dysplasia and intra-muscular as well as intra-osseous myxoma.

Myxoma of the Oral Soft Tissues: Report of a Case and Literature Review

Soft tissue myxomas are benign tumors of uncertain differentiation, arising in somatic soft tissues mostly within skeletal muscles, dermal and subcutaneous tissues, pelviperineal tissues, and adjacent to a large joint. They are classified as intramuscular myxoma, digital myxoma, superficial angiomyxoma, aggressive angiomyxoma, and juxta-articular myxoma. Although they are not encapsulated and may progressively invade the surrounding tissues, they have a benign behavior. Soft tissue myxomas affect persons of all ages although rarely children and young adolescents. Twenty percent of tumors grow intramuscularly (intramuscular myxoma); they are found frequently in the muscles of the thigh. In the maxillofacial regions soft tissue myxomas have been found in the skin and subcutaneous tissue, the fascia, the parotid gland, the larynx, the tonsils, and the ear. Soft tissue myxomas are found infrequently in the oral cavity. Only 24 cases were discovered in our review of the literature. We therefore thought it worthwhile to present a new case of soft tissue myxoma affecting the buccal mucosa.

Imaging of Paget's Disease and Fibrous Dysplasia of Bone

Imaging of Paget's Disease and Fibrous Dysplasia of Bone

Sonography of Intramuscular Myxomas

The objective of this study was to retrospectively review sonographic images of pathologically proven soft tissue myxomas to determine whether a sonographic correlate to the bright rim and bright cap signs described in the magnetic resonance imaging literature is present. The study group consisted of 6 patients with pathologically proven soft tissue myxomas (1 man and 5 women; age range, 41-72 years; mean, 56.5 years). The available sonographic images for each subject were retrospectively reviewed by 2 authors (L.F. and K.F.), with agreement reached by consensus. Among other findings, images were also reviewed for a peripheral rim of increased echogenicity (termed the "bright rim sign") and for the presence of a triangular hyperechoic area adjacent to at least one of the poles of the mass (termed the "bright cap sign"). The bright rim and bright cap signs were seen in 5 (83%) of the 6 myxomas. The single case without the bright cap sign was not the same case as the one lacking the bright rim sign. The sonographic bright rim and bright cap signs were associated with 5 (83%) of the 6 intramuscular myxomas. These findings correlate with their magnetic resonance imaging equivalents, which are well documented in the literature, due to muscle atrophy and adjacent fatty infiltration. Recognition of these features may assist in a more accurate sonographic diagnosis before biopsy.

Soft tissue myxoma: report of an unusual case located on the gingiva

Soft tissue myxoma of the oral cavity is rare. Only three cases of myxoma of gingiva have been reported in the literature. We present a case of soft tissue myxoma arising from the left maxillary adherent gingiva in a 42-year-old male [correction of female] patient. Histological examination showed spindle-shaped cells in a myxoid stroma. Immunohistochemical stains with S-100 protein were negative, while those with vimentin were positive. Clinical examination revealed a soft tissue mass, with tense elastic consistency on palpation. The overlying mucosa was normal and healthy. A clinical diagnosis of fibroma was given. Histological examination showed spindle-shaped and stellate cells, arranged in a myxoid fibrous stroma, with collagen fibres distributed uniformly. Scattered islands or strands of inactive odontogenic epithelium were present. On the basis of the histological and immunohistochemical findings, the final diagnosis was soft tissue myxoma. Further studies are necessary to clarify the origin and histogenesis of this lesion.