Mucinous Matrix Research Articles

Synovial myxosarcoma in the scapulohumeral joint of a dog: a case report.

A 12-year-old, 3kg spayed female mixed-breed dog was evaluated to assess a 1-year history of intermittent right forelimb lameness that did not have adequate response to nonsteroidal anti-inflammatory drugs. The radiographic study performed under sedation showed multifocal radiolucent areas affecting both the right humerus and scapula with focal soft tissue swelling; a CT scan confirmed the existence of an aggressive and invasive soft tissue mass affecting the scapulohumeral joint. Fine needle aspiration results suggested a low-grade synovial sarcoma and therefore a scapulectomy was performed. The biopsy showed spindle to stellated cells immersed in a basophilic and mucinous (myxoid) matrix with mild to moderate anisocytosis, moderate anisokaryosis, some binucleated cells and sporadic multinucleated cells. These findings are consistent with low-grade synovial myxosarcoma, a not well described synovial neoplasm that can mimic other commonly seen joint tumors or even septic arthritis on radiographs. The purpose of this case report is to describe the first reported synovial myxosarcoma affecting the scapulohumeral joint of a small dog.

Schwannoma: Another dermoscopic pitfall?

Schwannoma: Another dermoscopic pitfall?

A Rare Presentation of Thoracic Intramedullary Chordoma with Adjacent Bone Involvement: A Case Report and Review of the Literature

Abstract Introduction/Objective Chordoma originates from remnants of the embryonal notochord, and arise in bones anywhere along the spine and skull base. The most common location was thought to be the sacrum, followed by the clivus, and to a much lesser extent the rest of the spine. However, some studies have suggested an equal distribution among the skull base (32%), mobile spine (32.8%), and sacro-coccygeal bones (29.2%). Here we report a case of chordoma involving the thoracic spine. at the level of T2. Methods/Case Report A 63-year-old male with no significant past medical history who presented with 5-6 months of intermittent, bilateral lower extremity weakness and numbness in the trunk and lower extremities. MRI of the thoracic spine demonstrated a contrast enhancing mass at T2 vertebral level with spinal cord compression and adjacent bone destruction. T1-3 laminectomy with debulking of the tumor was performed. Microscopically, the tumor cells have a lobulated architecture and are composed of epithelioid cells arranged in cords, clusters or nests, embedded in a myxoid mucinous matrix. The epithelioid cells have a variably vacuolated cytoplasm ("physaliphorous" cells). The epithelioid cells are positive for CK AE1/3, Cam5.2, EMA and Brachyury (nuclear stain), and S100 (focal). These findings support a diagnosis of chordoma. Results (if a Case Study enter NA) N/A. Conclusion The most important and difficult differential diagnosis of chordoma is with well-differentiated chondrosarcoma. Although both chordomas and chondrosarcomas express S100, chondrosarcomas do not express cytokeratins, EMA or brachyury. Chordomas have an aggressive clinical course and poor outcome with local extension, recurrence and even metastasis. The treatment is en block surgical resection with adjuvant radiotherapy. The extent of the initial surgical resection is the most significant prognostic factor.

Immunohistochemical Characterization of a Duodenal Adenocarcinoma with Pulmonary, Hepatic and Parapatellar Metastases in a Common Marmoset (Callithrixjacchus)

An 11-year-old male common marmoset (Callithrix jacchus) presented with chronic, progressive weight loss and diarrhoea. Response to treatment with nutritional supplementation, antibiotics and immunosuppressants was modest and transient, and the animal was humanely euthanized. At necropsy, the proximal 8cm of small intestine was diffusely pale with transmural thickening. The lungs contained coalescing tan, firm nodules measuring up to 4mm in diameter. Histological examination revealed infiltrative mucinous adenocarcinoma of the duodenum with extensive metastases to the lungs, liver and left parapatellar adipose tissue. The mucinous matrix secreted by the primary and metastatic lesions was strongly periodic acid-Schiff positive. Warthin Starry staining for spirochaetes was negative. Pancytokeratin expression was attenuated in the primary tumour as well as in the metastases, which correlated to a poorly differentiated phenotype. To the authors' knowledge, this is the first report of a proximal duodenal adenocarcinoma with extensive metastatic disease in a common marmoset.

Mucinous Rectal Adenocarcinoma in a Urutu Snake (Bothrops alternatus)

A 15-year-old captive female Urutu snake (Bothrops alternatus) was presented with anorexia, apathy, dehydration, pale mucous membranes and marked enlargement of the caudal third of the coelomic cavity. Radiographs and ultrasonography were suggestive of faecal impaction or neoplasia. Post-mortem findings consisted of solid faecal impaction of the intestines, secondary to obstruction caused by a rectal tumour. Cytological examination of impression smears of the tumour revealed polygonal to round neoplastic cells arranged in clusters in an abundant extracellular mucinous matrix. Histologically, there was transmural growth of neoplastic epithelial cells arranged in small nests or individually and immersed in abundant extracellular mucin lakes. The neoplastic cells were immunopositive for cytokeratin. Clinical and pathological aspects indicated a diagnosis of mucinous rectal adenocarcinoma.

Primary conjunctival myxoma: case series and review of the literature.

Myxomas are benign soft tissue tumours resembling primitive mesenchyme. They rarely involve ocular structures, and have been recognized in the conjunctiva, eyelids, cornea, and orbit. The aim of this study was to investigate the clinicopathological features of seven patients with primary conjunctival myxoma seen at Dr Luis Sánchez Bulnes Hospital, an ophthalmological referral centre in Mexico City. We reviewed the clinical, histochemical and immunohistochemical studies of patients with documented myxoma of the conjunctiva diagnosed in our hospital. Seven conjunctival myxomas were retrieved from 5923 conjunctival biopsies (0.1%). The mean age of patients was 40 years, with a range of 27-51 years. Females were more frequently affected, and none of our patients had systemic disease. The left eye was involved in five cases, and most of the lesions were located in the bulbar conjunctiva. Histopathological examination revealed a benign tumour composed of spindle-shaped and stellate-shaped cells immersed within an abundant mucinous matrix with sparse vessels and reticulin fibres. Immunohistochemistry demonstrated positivity for vimentin and negativity for smooth muscle actin, SOX10 and GLUT1 in myxoma cells of all cases. S100 was found to be positive in four cases, and muscle-specific-actin in three cases. Conjunctival myxomas are uncommon tumours. For accurate diagnosis, histopathological examination is mandatory. The treatment of choice is surgical removal, and the prognosis is excellent.

Un cas de gliome chordoïde inhabituel : aspects immunohistochimiques et moléculaires et diagnostics différentiels

We report the case of a 63-year-old healthy patient who was admitted for surgery of a suprasellar tumor with extension to the optic chiasm responsible of visual disturbance. Histopathological examination revealed a tumoral proliferation composed of epithelioid cells without atypia arranged in cords in a mucinous matrix surrounded by some lymphocytic inflammatory infiltrates. On immunohistochemistry, the neoplastic cells strongly expressed GFAP and CD34, a weak expression of EMA, an expression of TTF1 without immunoreactivity for brachyury. Ki-67 labeling index was low around 1%. The diagnosis of chordoid glioma was made. Surprisingly, tumor cells expressed IDH1R132H but molecular analysis did not reveal any mutation of IDH1/2 genes. There was no expression of p53 but high overexpression of EGFR. Chordoid glioma is a rare and low-grade entity. The precise histogenesis remains debated. Our case is unusual because of the infiltration of the optic chiasm and because of the immunoexpression of IDH1R132H without underlying mutations of IDH1/2 genes.

EGF-FSH supplementation reduces apoptosis of pig granulosa cells in co-culture with cumulus-oocyte complexes

In cattle breeding, co-culture with granulosa cells (GCs) is one of the strategies to improve oocyte maturation and fertilization potential, but yields are still suboptimal due to GC apoptosis. We previously set up an in vitro co-culture system of cumulus-oocyte-complexes (COCs) anchored to GC multilayers adhering to the basal lamina (COCGs), in which GC apoptosis was inhibited by FSH supplementation. Here, we assessed the antiapoptotic effect of EGF (5 ng/ml-EGF5) alone or in synergism to FSH (50mU/ml-FSH50) on pig COCGs. COCG morphology, apoptotic rate, procaspase-8 and-9 expression levels and surface ultrastructure were determined. Results showed an increased % of apoptotic GCs in control and EGF5 (≈80%) respect to sampling (≈3%) and caspase-8 and -9 activation. In contrast, apoptotic cells were significantly reduced by FSH50 (≈35%) supplementation, with inactive Procaspase-8 and -9 highly expressed. The pro-survival effect of FSH was strengthened by EGF (EGF5+FSH50), as evidenced by a significant reduction of apoptosis (≈15%) and high expression levels of Procaspase-8 and -9. Ultrastructural analysis revealed that GC multilayers were characterized by round-to-ovoid cells connected each other and to the basal lamina by cytoplasmic projections. Microvilli shortening/thickening/reduction, cytoplasmic projection rarefaction, blebbing of apoptotic bodies and degenerating/atresic GCs were observed in control and EGF5 groups. FSH50 induced the formation of an abundant mucinous matrix, due to granulosa expansion. Blebs and atresic areas were rarely observed. In EGF5+FSH50 group, GCs were well-preserved, richly covered by microvilli and connected by numerous cytoplasmic projections. Degenerative phenomena were rarely observed. In conclusion, EGF in synergism with FSH seems to better counteract GC apoptosis in a co-culture of pig GC multilayers.

Macroscopic and histological investigation of guanaco footpads (Lama guanicoe, Müller 1776).

The surface of guanaco footpads is characterized by hairless skin with up to 4-mm-thick stratum corneum that protects from abrasion. The horny layer is pliable and elastic, and ensures firm contact with irregular ground. It is padded with a particular structure of the subcutaneous layer, the digital cushion. The flat cushions of each of the two digits are of elongated ovate shape, each about 45-mm long, up to 20-mm wide, and 8-mm thick. The cushions are lined by a 1-2-mm-thick capsule that resembles a tunica albuginea. The capsule consists of coarse collagen fibers, with elastic fibers absent. The cushion capsule and dermis approach each other, and fuse along a line that runs parallel to the longitudinal axes of cushion and digit. Loose connective tissue rich in elastic fibers and acidic glycosaminoglycans separates dermis and cushion capsule lateral to the narrow interconnecting zone. The cushion capsule encloses cloudy yellowish, gelatinous material. Microscopy shows bundles of elastic fibers in abundant mucinous matrix. Tightly gathered elastic bundles adjoin the inner surface of the capsule. Rough cords of elastic fibers branch out from there and traverse to the opposite side. The cushion is pressed flat, and elastic fibers are stretched when bearing weight. With relief of load, elastic fibers contract and reset the cushion's shape. Contractile cells are absent. A resistant capsule and easily malleable mucinous contents establish the functioning as a gel pad. Mucinous connective tissue between elastic fiber bundles contains abundant basophilic matrix. Hyaluronan, chondroitin sulfate, and dermatan sulfate are main matrix constituents. Spindle-shaped or stellate fibroblasts contain vimentin, S100 protein, and neuron specific enolase. Moprhology, staining characteristics and synthesis activities of these cells meet the criteria to be classified as myxoid cells. The connective tissue in guanaco digital cushions represents myxoid tissue.

Oligodendroglioma (WHO grade I) in a young epilepsy patient: A specific entity lying within the spectrum of dysembryoplastic neuroepithelial tumor?

We studied a frontal lobe subcortical cystic tumor that had been resected from a 13-year-old girl with a 3-year history of intractable partial seizure. Currently, more than 13 years after surgery, the patient remains recurrence-free and has no neurological deficits. Histological examination showed that the tumor was non-infiltrating and paucicellular with a mucinous matrix, and consisted of fairly uniform small cells with round to oval nuclei. Within the mucinous matrix, the tumor cells were often arranged in pseudorosettes around small blood vessels. Mitotic activity and necrosis were absent, with a Ki-67 labeling index of <1%. Based on the immunohistochemical and ultrastructural findings, the constituent tumor cells were considered to be those of oligodendroglioma, including mini-gemistocytes and gliofibrillary oligodendrocytes. No neuronal elements were identified. Features of cortical dysplasia (FCD Type 1) were evident in the cortex covering the lesion. The surrounding white matter also contained a significant number of ectopic neurons. The entire pathological picture appeared to differ somewhat from that of ordinary oligodendroglioma (WHO grade II). Considering the clinical and pathological features, the present unusual oligodendroglioma appeared to represent a previously undescribed form of oligodendroglioma (WHO grade I) lying within the spectrum of dysembryoplastic neuroepithelial tumor (DNT; WHO grade I). Simultaneously, the present oligodendroglioma also raises the question of whether or not oligodendrocyte-like cells of DNTs truly show neurocytic differentiation.

Malignant Dysembryoplastic Neuroepithelial Tumour in a Zebrafish (Danio rerio)

Neuroectodermal tumours in man, including medulloblastoma, medulloepithelioma, neuroblastoma, esthesioneuroblastoma, primitive neuroectodermal tumour and dysembryoplastic neuroepithelial tumour, typically occur in children and young adults. These tumour types are occasionally observed in juvenile and adult zebrafish (Danio rerio) either as induced tumours in carcinogen-exposed zebrafish or as an incidental finding in zebrafish≥2years of age. An adult zebrafish submitted for routine histological examination was sent for a second opinion consultation after an uncharacteristic brain mass was identified. Microscopically, the expansile and infiltrative extracortical mass arising from the cerebellum had a diffuse microcystic pattern with solid hypercellular regions occupying 80% of the extrameningeal space and effacing the endomeninx and significantly displacing the metencephalon. The mass was composed of dense sheets of oligodendrocyte-like cells, random neurons and pseudocysts containing 'floating neurons' within a scant mucinous matrix. Neoplastic cells demonstrated positive perinuclear and intracytoplasmic expression of S-100. Malignant dysembryoplastic neuroepithelial tumour was diagnosed based on the histological features of the brain mass, which were indistinguishable from the human tumour. To our knowledge, this is the first report of a dysembryoplastic neuroepithelial tumour in a zebrafish.

Malignant peripheral nerve sheath tumor in a cat with nodal and pulmonary metastases

Peripheral nerve sheath tumors in domestic cats are infrequently reported and are often locally invasive. An 11-year-old Domestic Shorthair cat was originally diagnosed with a right maxillary benign peripheral nerve sheath tumor at incisional biopsy. At necropsy, the neoplasm had features of malignancy including metastases to the regional lymph nodes and lung. Histologically, the neoplasm contained 2 distinct regions: spindle cells arranged in dense interwoven bundles with Antoni A areas and Verocay bodies and Antoni B regions with loosely arranged spindle cells separated by a mucinous matrix. Immunohistochemically, the neoplastic cells in the primary mass and right mandibular lymph node were strongly positive for vimentin, S-100, and glial fibrillar acidic protein. The neoplastic cells within the lung were strongly positive for vimentin and weakly positive for S-100 and glial fibrillar acidic protein.

A Grade IV Glioblastoma with an Oligodendroglial Component (GBM-O) in a Horse

A 4-year-old Dutch warmblood mare was presented with a 10-month history of ataxia and proprioceptive deficits. Computed tomography defined a large, non-contrast enhancing mass in the left cerebral hemisphere. Necropsy examination revealed a tumour that effaced much of the piriform and temporal lobes. Microscopically the lesion was classified as a grade IV glioblastoma with an oligodendroglial component (GBM-O). The tumour was composed of highly pleomorphic cells organized in different patterns within a fibrillary stroma. There were multiple foci of necrosis. At the periphery of the tumour neoplastic oligodendroglioma-like cells were embedded in an extracellular mucinous matrix. Most neoplastic cells were strongly immunoreactive for glial fibrillary acidic protein; however, the oligodendroglioma cells did not express this marker. Cells forming microvascular proliferations were positively labelled for expression of factor VIII and smooth muscle actin. All neoplastic cells were negative for Neu-N and synaptophysin. The proliferation index was up to 5%. All neoplastic cells and normal brain tissue from the horse were uniformly negative for expression of epidermal growth factor receptor (EGFR), EGFR vIII mutant and the phosphatase and tensin homologue (PTEN) compared with positive control human GBM tissue. To our knowledge this is the first report of a GBM-O in the horse.

Carney complex: A clinicopathologic and molecular biological study of a sporadic case, including extracutaneous and cutaneous lesions and a novel mutation of the PRKAR1A gene

Carney complex (CNC) is an autosomal dominant disorder associated with multiple neoplasms. We report a case of a 40-year-old Caucasian man with a sporadic form of CNC. This is a clinicopathologic description and molecular biological study with an emphasis on histopathologic findings. The patient presented with multiple cutaneous myxomas, cardiac myxomas, and spotty pigmentation at typical sites. Additionally, a blue nevus, a lipoma, multiple calcifications in both testes, and hypoechogenic areas suspected of being adenomas in the thyroid gland were found. Microscopically, the 2 cardiac and 6 cutaneous myxomas studied manifested a typical appearance, being composed of scattered polygonal, stellate, plump and/or spindle cells in a mucinous matrix containing small, sometimes dilated blood vessels. Of the 6 cutaneous myxomas, only in one lesion was there an abnormal epithelial component (tiny basaloid buds and a horn cyst). Molecular biologic study revealed a heterozygous shift mutation c.796dupA in exon 10 of the PRKAR1A gene. Physical examination and genetic testing of family members (both parents and two brothers) for the PRKAR1A mutation were negative, as was analysis of the peripheral blood of 110 randomly selected, unrelated healthy individuals for the above mutation. These findings suggest sporadic disease and a novel mutation in our patient. None. Herein we report a case of sporadic CNC in which a novel mutation in PRKAR1A was identified.

What is your diagnosis? Vertebral mass in a dog

A 1-year-old, castrated male, mixed-breed dog was presented for sporadic episodes of kyphosis, tremors, and vocalizing. On neurologic examination, the lesion was localized to spinal cord segments T3-L3. Magnetic resonance imaging of the spine showed an expansile mass occupying most of the ventral aspect of the spinous process of T6. Fine-needle aspirates of the mass were examined cytologically. A moderately cellular population of pleomorphic spindle cells and abundant mucinous matrix were observed. The cytologic diagnosis was spindle cell neoplasia, with myxosarcoma and fibrosarcoma as the primary differential diagnoses. The dog was euthanized. Histopathologic evaluation of the mass and surrounding tissue confirmed a low-grade spindle cell sarcoma, with severe compressive myelopathy and mild neutrophilic inflammation. The neoplastic cells stained positive for mucopolysaccharides with Alcian blue, resulting in a final diagnosis of low-grade (grade 1) myxosarcoma. Fine-needle aspiration was useful in making a preliminary diagnosis of myxosarcoma in this case. Myxosarcoma should be included in the differential diagnosis for a vertebral mass in a young dog.

Plexiform angiomyxoid myofibroblastic tumour: differential diagnosis of gastrointestinal stromal tumour in the stomach

Mesenchymal tumours other than gastrointestinal stromal tumours are rare in the stomach. Nevertheless it is important to incorporate them into the differential diagnosis. Plexiform angiomyxoid myofibroblastic tumour is a recently...

Myositis Ossificans Associated With Subclinical Idiopathic Thrombocytopenic Purpura: Report of a Case

A 56-year-old healthy man noticed a stony-hard mass gradually grown in his neck for more than a 6-year period. Examinations revealed 2 additional calcified masses at the esophageal hiatus and retroperitoneum. The cut-surface of the operative specimens showed fibrous and mucinous appearance with many calcified islands. Microscopically, the lesion showed a gradual transform from the central immature area with fibroblasts and mucinous matrix, to the peripheral mature area with woven bone and osteoclasts (zoning phenomenon), which was compatible with the features of mature myositis ossificans. Myositis ossificans is an isolated, benign, nonneoplastic disease typically found in adolescence, after traumatic episode. Similar lesions have been found in the major musculature of patients with increased bleeding tendency. In the present case, idiopathic thrombocytopenic purpura was found to be associated and was suggested to be involved in initiating myositis ossificans. Still, it is extremely rare to observe lesions in these sites synchronously.

A mammary gland adenomyoepithelioma in a C57BL/6 mouse

Mammary gland adenomyoepitheliomas are benign complex mammary gland tumors composed of neoplastic cells of epithelial and myoepithelial origins, described in many species (humans, dogs, cats, rats) and rarely in mice. We report here an adenomyoepithelioma in a C57BL/6 female mouse. Histologically, tubes and cords formed by neoplastic epithelial cells were separated by bundles of neoplastic myoepithelial cells in a clear and partially mucinous matrix. The tumor displayed characteristics of a benign neoplastic proliferation with a compressive growth pattern, and moderate cellular pleomorphism and mitotic index. At immunohistochemistry, the epithelial cells were strongly cytokeratin positive; the myoepithelial cells were weakly cytokeratin positive and strongly smooth muscle actin positive. This is to our knowledge, the first report of a mammary gland adenomyoepithelioma in a C57BL/6 mouse.

Cervical Chondroid Chordoma in a Shetland Sheep Dog

A spayed female Shetland sheep dog aged 12 years was presented for examination with ataxia and hindlimb paralysis. Extradural spinal cord compression was found at the level of vertebrae C6-C7 by radiography and myelocomputed tomography. A jelly-like mass (0.6 x 1.3 cm) was removed surgically. Histopathological findings were characterized by proliferation of vacuolated polygonal cells (physaliphorous cells) in a mucinous matrix and the presence of chondroid tissue shown immunohistochemically to express S-100. The physaliphorous cells were immunolabelled strongly for vimentin and S-100, and weakly for cytokeratin. A diagnosis of canine cervical chondroid chordoma was made. This is considered to be the first report of a chondroid chordoma originating from the cervical region of the spine in the dog.

Undifferentiated Perivascular Cells in Myxoid Mesenchymal Tumors: An Ultrastructural Study

The current WHO classification of soft tissue tumors is based on the lineage of differentiation of the proliferating cells. Since mature mesenchymal cells have a broad phenotypic plasticity it has been considered unnecessary to recur to a hypothetical stem cell to explain the origin of these neoplasms. In spite of this assumption, the target cell of the oncogenic mutations in mesenchymal tumors is still a controversial item. Myxoid mesenchymal tumors constitute a heterogeneous group of neoplasms sharing in an ample mucinous matrix that separates neoplastic cells and facilitates their single submicroscopic study under electron microscopy examination. The authors have studied, by electron microscopy, 74 myxoid mesenchymal tumors, including a large variety of nosologic entities, to assess their madurational gradient. In 43 of 74 cases, a common element has been found: medium-sized cells, with high nucleo-cytoplasmic ratio, lacking lineage specific features, which were arranged around the capillary vessels. In some cases, the authors were able to demonstrate gradual differentiation in these cells, as they moved away from the vessels. These features support the hypothesis that at least some mesenchymal tumors originate from perivascular undifferentiated cells. In addition, the findings might contribute to define both topographic and morphologic characteristics of adult stem mesenchymal cells.