Cutaneous Myxoid Research Articles

Oral Focal Mucinosis, an uncommon entity: Report of three cases

<h3>Introduction</h3> Oral focal mucinosis (OFM) is a rare soft tissue lesion of unknown etiology and it is considered the oral counterpart of cutaneous focal mucinosis or cutaneous myxoid cyst. The pathogenesis of OFM may be explained by fibroblast overproduction of hyaluronic acid. Clinically, it appears as painless swelling of the same color as the surrounding mucosa. The gingiva is the most common site and the diagnosis is established only by histopathological and immunohistological examination. <h3>Materials and Methods</h3> We present three cases of gingival oral focal mucinosis. Case 1: 40 year-old womatn with painless and slow-growing swelling of two years of evolution at buccal mandibular gingiva, between lateral incisor and canine. The lesion measured 2 cm. and exhibited the same color as the overlying mucosa; there was not radiographic involvement. Case 2: 48 year-old female with a hard palatal swelling. The lesion was sessile, firm, and not color change Case 3: 36 year-old male with a painless and same color as the normal mucosa gingival swelling. The lesion was between central and lateral right incisors, it was sessil and measured 1.5 cm Results In the three cases excisional biopsy was performed and histopathologically they shown the proliferation of elongated and mucinous material in a myxoid matrix. This myxoid stroma was surrounded by collagenous fibrous connective tissue and covered with squamous epithelium. Histochemical staining with Alcian blue was positive in the mucinous areas Conclusions These three cases are significant clinically as well as histopathologically. The lesions clinically mimic an ossifying peripheral fibroma or a fibroma. Thus, the histopathological and histochemical findings with special stains, such as Alcian blue, are determinant to establish the diagnosis of these conditions. Treatment is the complete surgical excision and its recurrence is not reported.

Superficial Angiomyxomas Frequently Demonstrate Loss of Protein Kinase A Regulatory Subunit 1 Alpha Expression: Immunohistochemical Analysis of 29 Cases and Cutaneous Myxoid Neoplasms With Histopathologic Overlap.

Superficial angiomyxomas (SAMs) are benign cutaneous tumors that arise de novo and in the setting of the Carney complex (CC), an autosomal dominant disease with several cutaneous manifestations including lentigines and pigmented epithelioid melanocytomas. Although most SAM do not pose a diagnostic challenge, a subset can demonstrate histopathologic overlap with other myxoid tumors that arise in the skin and subcutis. Traditional immunohistochemical markers are of limited utility when discriminating SAM from histopathologic mimics. Since protein kinase A regulatory subunit 1 alpha (PRKAR1A) genetic alterations underlie most CC cases, we investigated whether SAM demonstrate loss of PRKAR1A protein expression by immunohistochemistry. In our series, 29 SAM, 26 myxofibrosarcoma, 5 myxoid dermatofibrosarcoma protuberans, 11 superficial acral fibromyxomas, and 18 digital mucous cysts were characterized. Of the 29 SAM examined in this study, 1 was associated with documented CC in a 5-year-old girl. SAM tended to arise in adults (mean 49.7 y; range: 5 to 87 y). Loss of PRKAR1A was seen in 55.2% of cases (16/29) and had a male predilection (87.5%, 12/16). PRKAR1A-inactivated SAM demonstrated significant nuclear enlargement (100%, 16/16 vs. 23.1%, 3/13), multinucleation (81.3%, 13/16 vs. 23.1%, 3/13), and presence of neutrophils (43.8%, 7/16 vs. 0%, 0/13). In contrast, PRKAR1A was retained in all cases of myxofibrosarcoma (100%, 26/26), myxoid dermatofibrosarcoma protuberans (100%, 5/5), superficial acral fibromyxomas (100%, 11/11), and digital mucous cyst (100%, 18/18). Taken together, PRKAR1A loss by immunohistochemistry can be used as an adjunctive assay to support the diagnosis of SAM given the high specificity of this staining pattern compared with histopathologic mimics.

Clinicopathological study of oral focal mucinosis: a retrospective case series.

BackgroundOral focal mucinosis (OFM) is a rare soft tissue lesion of unknown etiology that exhibits tumor-like growth. It is considered the oral counterpart of cutaneous focal mucinosis or cutaneous myxoid cyst. This is a retrospective study of oral OFM diagnosed over a period of 42 years at an oral pathology service.Material and MethodsClinical, histopathological and immunohistochemical data were analyzed. Alcian blue staining and S-100 immunohistochemistry were performed.ResultsEleven cases were retrieved (4:1 female-to-male ratio). The mean age was 44 years. The gingiva was the most affected site. The main clinical presentation was sessile or pedunculated lesions of fibrous or hyperplasic appearance, most of them asymptomatic. Positive Alcian blue staining and absence of S-100 protein were observed in all specimens, which supported the histological diagnosis of OFM. Surgical excision was the treatment of choice.ConclusionsAlthough rare, this study supports the inclusion of OFM in the differential diagnosis of intraoral myxoid lesions. Key words:Oral focal mucinosis, myxomatous lesion, connective tissue diseases, diagnosis.

Telocytes as a Source of Progenitor Cells in Regeneration and Repair Through Granulation Tissue.

This review outlines the role of CD34+ stromal cells/telocytes (CD34+ SC/TCs) in repair and considers the following issues. Firstly, the conceptual aspects of repair, including regeneration and repair through granulation tissue (RTGT) as two types of repair, RTGT stages (inflammatory, proliferative, and remodeling), and tissue in repair as a substrate to assess the in vivo behavior of activated CD34+ SC/TCs. Subsequently, current knowledge of CD34+ SC/TCs, such as identification, characteristics, and functions, as well as possible stages (quiescent and activated) are taken into account. We then consider the role in regeneration of quiescent CD34+ SC/TCs (in unperturbed physiological conditions) as a nurse of stem cells (e.g., in the heart, skin, respiratory tree, gastrointestinal tract, liver, eye, and choroid plexus). Special attention is paid to the characteristics of activated CD34+ SC/TCs and the overlapping steps of activation with and without loss of CD34 expression and with and without gain of αSMA expression. With this contribution, we establish the role of CD34+ SC/TCs as progenitor cells and as a source of fibroblasts and myofibroblasts in repair through granulation tissue, fibrosis, and tumor stroma. Activated CD34+ SC/TCs in encapsulation and other processes (e.g., Reinke's edema, cutaneous myxoid cyst, mixomatous mitral valve degeneration, and fibrous papula of the face) are also outlined. Finally, similarities between modifications of CD34+ SC/TCs during in vivo activation and of multipotent mesenchymal stromal/stem cells in culture are examined in order to correlate the growing literature on CD34+ SC/TCs and the exponential research in cultured mesenchymal stromal/stem cells.

再発と多発性肺転移を伴った皮膚の骨外性粘液性軟骨肉腫の犬の一例

再発と多発性肺転移を伴った皮膚の骨外性粘液性軟骨肉腫の犬の一例

Isolated Subcutaneous Metastasis of Myxoid Liposarcoma: A Case Report

Background: Subcutaneous metastatic liposarcomas are rare. Differentiation between primary tumors and metastasis of a single liposarcoma represents the main difficulty in diagnosis. In this article, we describe the first report of Subcutaneous metastasis to the neck in the right level IB from liposarcoma originating in the thigh. Case presentation: The present 19-year-old women presented to a complaint of a rapidly growing mass, of the posterior loge of the right thigh with poor definition of adjacent structures, of 9 months’ duration measuring 188 × 97 mm. Microscopic examination of the mass following excision revealed a myxoid liposarcoma. A wide surgical resection was performed, and margins were negative. At this time, the patient showed no metastatic disease and underwent a complementary treatment including irradiation of the right thigh at a dose of 50 Gy delivered in 25 fractions over 38 days. There weren’t any local recurrence or metastases on her 12 months follow up until May 2015 when she presented with a mass in the neck (right level IB). An excisional biopsy was performed by an in June 2015 revealing a myxoid liposarcoma imposing a large re-excision of the tumor bed with 3cm free tumor margin. Microscopic examination of the surgical specimen found clear margins without an involvement of the skin. Differentiation between primary tumors and metastasis of a single liposarcoma was very difficult. The results of the disease extension workup showed no sigh of other metastases no local recurrence until now. Conclusion: To our knowledge, no case of cutaneous metastatic myxoid liposarcoma has been reported until now.

Cutaneous myxoid cyst on the sclerotic finger in a patient with diffuse systemic sclerosis

Skin tumors occurring on the scleroderma fingers are rarely seen. Swollen fingers are hallmarks of systemic sclerosis, and mucin deposition in the lesional skin is a constant feature in systemic sclerosis. Here we describe a case of cutaneous myxoid cyst on the flexor aspect of the sclerotic fingers in a patient with severe diffuse cutaneous systemic sclerosis. Cutaneous myxoid cyst is a relatively common benign tumor; however, cases of cutaneous myxoid cysts developing on the scleroderma fingers have not been reported to date. Mucin deposition in the sclerotic skin may be a predisposing factor in the induction of myxoid cyst on the scleroderma finger in our patient.

Oral focal mucinosis: a case report and review of the literature

Oral focal mucinosis is a rare clinicopathological entity that represents the mucosal counterpart of cutaneous focal mucinosis or cutaneous myxoid cyst. The aetiology is unknown. Oral focal mucinosis is most common in young adults. The gingiva is the most common site and the hard palate is the second most common location. A 19-year-old male patient presented with a 2 cm painless mass localized to the palatal side. An excisional biopsy was taken and sent for histopathologic evaluation to our department. Histopathologic findings were a well-circumscribed lesion composed of myxomatous connective tissue that contained bipolar, fusiform or stellate shaped fibroblasts. The aim of this study was to bring oral focal musinosis diagnosis to the attention of clinicians and pathologists when considering the differential diagnosis of gingival and palatal nodules.

Oral focal mucinosis: Case report

Oral focal mucinosis (OFM) is an uncommon disease of unknown aetiology. It is considered to be the oral counterpart of cutaneous focal mucinosis and cutaneous myxoid cyst and it is characterized by a focal myxoid degeneration of the connective tissue. A preoperative diagnosis is almost impossible, and the clinical suspicion, usually made by exclusion, must be confirmed by a bioptic examination followed by histological observations.The Authors report a case of focal oral mucinosis diagnosed and treated in a male adult patient.

Cutaneous myxoid solitary fibrous tumour

Cutaneous myxoid solitary fibrous tumour

Cutaneous myxoid fibroblastoma

Cutaneous myxoid fibroblastoma is an extremely rare soft tissue tumor, diagnosed based on histopathology. The histopathological triad for the diagnosis of the disease includes 1) spindle-shaped and/or stellate cells with elongated nuclei and eosinophilic cytoplasm 2) distribution of cell in amyxoid stroma containing very fewcollagen fibers 3) cellular atypiawithout marked nuclear polymorphism in some cells. To date, only single cases of this disease have been reported. We report a new case and discuss the disease.

Cutaneous leiomyosarcoma and intramuscular myxoid liposarcoma in a patient with hereditary retinoblastoma

Cutaneous leiomyosarcoma and intramuscular myxoid liposarcoma in a patient with hereditary retinoblastoma

Multiple cutaneous myxoid cysts with transepidermal elimination

Multiple cutaneous myxoid cysts with transepidermal elimination

Myxofibrosarcoma presenting in the skin: clinicopathological features and differential diagnosis with cutaneous myxoid neoplasms.

Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) is one of the most common fibroblastic sarcomas in the older patient, where it can sometimes present with anatomically deceptive boundaries. Myxofibrosarcoma is now fully characterized as a distinctive and definable pathologic entity. Clinically there is a tendency for predominantly subcutaneous, multinodular, diffusely infiltrative growth, which may extend to the overlying dermis and present as a cutaneous lesion. Histologically myxofibrosarcoma comprises a spectrum ranging from hypocellular low-grade myxoid to high-grade pleomorphic sarcoma. We report herein 6 cases of myxofibrosarcoma each with dermatological presentation as a cutaneous nodule. The dermal component in each of the lesions was low- to intermediate-grade and predominantly myxoid resulting in confusion with benign myxoid neoplasms in small biopsy specimens. The purpose of this series is to focus the attention of workers in dermatology on a subject rarely discussed in dermatopathology literature: the cutaneous presentation of myxofibrosarcoma and the potential for clinical and histologic misinterpretation, as benign dermal lesions.

Primary cutaneous myxoid melanoma: Immunohistologic clues to a difficult diagnosis

We describe a 25-year-old man with a primary cutaneous myxoid malignant melanoma and xeroderma pigmentosum. Histologically, the tumor had a lentiginous intraepidermal component and a dermal myxoid nodule containing fusiform cells with hyperchromatic nuclei and nuclear pseudoinclusions. Immunohistochemically, the tumor cells were positive for S-100 protein in both the epidermis and the dermis and did not stain with HMB-45, AE1-AE3, MNF 116, antiactin, or anti-p53 protein. Although this tumor is rare, it should be considered in the differential diagnosis of cutaneous myxoid lesions.

Cutaneous Myxoid Fibroblastoma

A fibroblastic skin tumor with a myxoid matrix is reported that cannot be easily classified as one of the well-known entities of fibrous/fibrohistiocytic and myxoid skin tumors. A 27-year-old white woman presented with a reddish, dome-shaped cutaneous nodule 8 mm in diameter on the left popliteal fossa that had developed spontaneously within the preceding 2 years. There was no sign of recurrence 30 months after excision. Light microscopic examination showed a well-circumscribed tumor confined to the upper dermis and consisting of stellate and spindle-shaped cells arranged loosely in a fascicular pattern resembling tissue cultures of fibroblasts. There were almost no collagen bundles between tumor cells, and Mowry's staining showed large amounts of glycosaminoglycans. Immunohistochemical studies of the tumor cells showed reactivity only to vimentin, whereas markers of histiocytes, dermal dendrocytes, and neurogenic and myogenic differentiation were negative. By electron microscopy, the majority of tumor cells contained elliptical nuclei, but some tumor cells had conspicuous multisegmented nuclei with several large and small nuclear segments connected by thin nuclear bridges (labyrinth nuclei). Single fibrils were found within the interstitium; collagen fibers were rare. Histological and ultrastructural examinations identified tumor cells as fibroblasts. High cellularity distinguishes this tumor from cutaneous myxoma. We conclude that this lesion represents a newly recognized tumor of fibroblastic origin. The name cutaneous myxoid fibroblastoma is proposed.

Oral focal mucinosis

Oral focal mucinosis (OFM) is an uncommon clinicopathological entity which is considered to be the oral counterpart of cutaneous focal mucinosis and/or cutaneous myxoid cyst. It is comprised of a clinically elevated mass with a histological picture of localized areas of myxomatous connective tissue. The present study adds 15 new cases of OFM to the literature and analyzes their clinical, histological and histochemical features. Most of the lesions were located in the gingiva and alveolar mucosa. The nature of the lesion is unclear and it is suggested that the mucinous accumulation is the result of fibroblastic overproduction of hyaluronic acid.

Cutaneous Mucinosis of Infancy

Cutaneous mucinosis is a term that has been used to describe a group of diseases or conditions in which accumulation of mucin in the skin is a prominent feature. The cutaneous mucinoses includes myxedema (both diffuse and localized), lichen myxedematosus (papular mucinosis), lipoid proteinosis, follicular mucinosis, cutaneous focal mucinosis, cutaneous myxoid cyst, and others. All of these diseases share distinct histologic features. I examined a 16-month-old infant with a case of cutaneous mucinosis that had unique clinical and histologic features, unlike any of the known mucinoses.

Oral focal mucinosis: A clinicopathologic and histochemical study of eight cases

Eight cases of the oral counterpart of cutaneous focal mucinosis and/or cutaneous myxoid cyst are reported, and their histologic and histochemical findings are discussed. On the basis of these findings, it is suggested that the lesions are identical and differ only in their anatomic location. It appears likely that most, if not all, cases diagnosed as oral soft-tissue myxomas are in reality oral lesions of the entity described as cutaneous focal mucinosis. It is postulated that focal mucinosis develops because of a fibroblastic overproduction of hyaluronic acid due to an unknown stimulus. The lesion is treated by surgical excision and has no tendency to recur.

Myxoid Cysts

The digital disturbances pictured are examples of an uncommon cutaneous growth which causes concern either by the production of local discomfort or by disturbance of the normal pattern of nail growth. These cystic lesions occur most commonly on terminal parts of the fingers, but they can also be found on other areas of the hands, or occasionally on the feet. Simple observation is usually all that is necessary for diagnosis of these cutaneous myxoid cysts. In most instances, a firm, single, fluctuant nodule is visible and palpable (Fig 1). Sometimes the cyst itself is not apparent, but the resultant disturbance in nail-forming epithelium can be recognized by a furrow in the nail plate (Fig 2). A clear, thick, syrup-like material can often be expressed from the incised lesion. These lesions were once thought to be cysts which extended from a joint capsule or tendon sheath into the dermis (and therefore