Ectomesenchymal chondromyxoid tumor

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Ectomesenchymal chondromyxoid tumor (ECT) is a benign intraoral tumor with presumed origin from undifferentiated (ecto)mesenchymal cells. [1] There are some who think it is a myoepithelial tumor type. [2]

Myoepithelioma of the head and neck sweat gland neoplasm that is composed of outgrowths of myoepithelial cells from a sweat gland

Myoepithelioma of the head and neck, also myoepithelioma, is a salivary gland tumour of the head and neck that is usually benign.

Contents

Controversies about origin

Neural crest

Neural crest cells are a temporary group of cells unique to chordates of the group Cristozoa that arise from the embryonic ectoderm cell layer, and in turn give rise to a diverse cell lineage—including melanocytes, craniofacial cartilage and bone, smooth muscle, peripheral and enteric neurons and glia.

Signs and Symptoms

Patients present with a painless, slow-growing mass usually within the tongue (most commonly the anterior dorsal tongue). There is an intact surface epithelium. [1] [5] [6]

Management

Pathology findings

Macroscopic

Microscopic Pathology

Skeletal muscle one of three major muscle types

Skeletal muscle is one of three major muscle types, the others being cardiac muscle and smooth muscle. It is a form of striated muscle tissue, which is under the voluntary control of the somatic nervous system. Most skeletal muscles are attached to bones by bundles of collagen fibers known as tendons.

Nerve enclosed, cable-like bundle of axons in the peripheral nervous system

A nerve is an enclosed, cable-like bundle of nerve fibres called axons, in the peripheral nervous system. A nerve provides a common pathway for the electrochemical nerve impulses called action potentials that are transmitted along each of the axons to peripheral organs or, in the case of sensory nerves, from the periphery back to the central nervous system. Each axon within the nerve is an extension of an individual neuron, along with other supportive cells such as Schwann cells that coat the axons in myelin.

Stellate cell

Stellate cells are any neuron in the central nervous system that have a star-like shape formed by dendritic processes radiating from the cell body. Many Stellate cells are GABAergic and are located in the molecular layer of the cerebellum. Stellate cells are derived from dividing progenitors in the white matter of postnatal cerebellum. Dendritic trees can vary between neurons. There are two types of dendritic trees in the cerebral cortex, which include pyramidal cells, which are pyramid shaped and stellate cells which are star shaped. Dendrites can also aid neuron classification. Dendrites with spines are classified as spiny, those without spines are classified as aspinous. Stellate cells can be spiny or aspinous, while pyramidal cells are always spiny. Most common stellate cells are the inhibitory interneurons found within the upper half of the molecular layer in the cerebellum. Cerebellar stellate cells synapse onto the dendritic arbors of Purkinje cells and send inhibitory signals. Stellate neurons are sometimes found in other locations in the central nervous system; cortical spiny stellate cells are found in layer IVC of the V1 region in the visual cortex. In the somatosensory barrel cortex of mice and rats, glutamatergic (excitatory) spiny stellate cells are organized in the barrels of layer 4. They receive excitatory synaptic fibres from the thalamus and process feed forward excitation to 2/3 layer of V1 visual cortex to pyramidal cells. Cortical spiny stellate cells have a 'regular' firing pattern. Stellate cells are chromophobes, that is cells that does not stain readily, and thus appears relatively pale under the microscope.

Immunohistochemistry

Ancillary Studies

Differential diagnoses

The tumor is quite unique, but other tumors are considered in the differential diagnosis histologically. They included pleomorphic adenoma, myoepithelioma, myxoid neurofibroma, neurothekeoma (nerve sheath myxoma), chondroid choristoma, extraskeletal myxoid chondrosarcoma, focal oral mucinosis, and an ossifying fibromyxoid tumor of soft parts. [1]

Epidemiology

Exceedingly rare, this tumor develops in a wide age range, although often in young patients without a sex predilection. The vast majority develop within the anterior dorsal tongue, [1] with palate and base of tongue rarely affected. [9]

Related Research Articles

Gastrointestinal stromal tumor Human disease

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. GISTs arise in the smooth muscle pacemaker interstitial cell of Cajal, or similar cells. They are defined as tumors whose behavior is driven by mutations in the KIT gene (85%), PDGFRA gene (10%), or BRAF kinase (rare). 95% of GISTs stain positively for KIT (CD117). Most (66%) occur in the stomach and gastric GISTs have a lower malignant potential than tumors found elsewhere in the GI tract.

Rhabdomyoma benign mesenchymal tumor arising from skeletal or cardiac muscle

A rhabdomyoma is a benign tumor of striated muscle. Rhabdomyomas may be either "cardiac" or "extra cardiac". Extracardiac forms of rhabdomyoma are sub classified into three distinct types: adult type, fetal type, and genital type.

Hemangioendothelioma group of vascular neoplasms

Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy.

Pleomorphic adenoma gastrointestinal benign neoplasm that is a located in the salivary glands

Pleomorphic adenoma is a common benign salivary gland neoplasm characterised by neoplastic proliferation of parenchymatous glandular cells along with myoepithelial components, having a malignant potentiality. It is the most common type of salivary gland tumor and the most common tumor of the parotid gland. It derives its name from the architectural Pleomorphism seen by light microscopy. It is also known as "Mixed tumor, salivary gland type", which refers to its dual origin from epithelial and myoepithelial elements as opposed to its pleomorphic appearance.

Canalicular adenoma Benign salivary gland tumor

Canalicular adenoma is a benign, epithelial salivary gland neoplasm arranged in interconnecting cords of columnar cells. This is a very rare benign neoplasm, that makes up about 1% of all salivary gland tumors, or about 4% of all benign salivary gland tumors.

Submucosa

The submucosa is a thin layer of tissue in various organs of the gastrointestinal, respiratory, and genitourinary tracts. It is the layer of dense irregular connective tissue that supports the mucosa and joins it to the muscular layer, the bulk of overlying smooth muscle.

Myoepithelial cells are cells usually found in glandular epithelium as a thin layer above the basement membrane but generally beneath the luminal cells. These may be positive for alpha smooth muscle actin and can contract and expel the secretions of exocrine glands. They are found in the sweat glands, mammary glands, lacrimal glands, and salivary glands. Myoepithelial cells in these cases constitute the basal cell layer of an epithelium that harbors the epithelial progenitor. In the case of wound healing, myoepithelial cells reactively proliferate. Presence of myoepithelial cells in a hyperplastic tissue proves the benignity of the gland and, when absent, indicates cancer. Only rare cancers like adenoid cystic carcinomas contains myoepithelial cells as one of the malignant component.

Segmental odontomaxillary dysplasia is a painless, unilateral enlargement of the upper jaw. The cause is unknown, and the disease affects the jaws, teeth, and adjacent soft tissue. The premolars may be congenitally missing, and the primary teeth may be smaller than usual. It is a relatively recent discovery.

The calcifying epithelial odontogenic tumor (CEOT), also known as a Pindborg tumor, is an odontogenic tumor first recognized by the Danish pathologist Jens Jørgen Pindborg in 1955. It was previously described as an adenoid adamantoblastoma, unusual ameloblastoma and a cystic odontoma. Like other odontogenic neoplasms, it is thought to arise from the epithelial element of the enamel origin. It is a typically benign and slow growing, but invasive neoplasm.

Nasopalatine duct cyst

The nasopalatine duct cyst (NPDC) occurs in the median of the palate, usually anterior to first molars. It often appears between the roots of the maxillary central incisors. Radiographically, it may often appear as a heart-shaped radiolucency. It is usually asymptomatic, but may sometimes produce an elevation in the anterior portion of the palate. It was first described by Meyer in 1914.

Neprilysin protein-coding gene in the species Homo sapiens

Neprilysin, also known as membrane metallo-endopeptidase (MME), neutral endopeptidase (NEP), cluster of differentiation 10 (CD10), and common acute lymphoblastic leukemia antigen (CALLA) is an enzyme that in humans is encoded by the MME gene. Neprilysin is a zinc-dependent metalloprotease that cleaves peptides at the amino side of hydrophobic residues and inactivates several peptide hormones including glucagon, enkephalins, substance P, neurotensin, oxytocin, and bradykinin. It also degrades the amyloid beta peptide whose abnormal folding and aggregation in neural tissue has been implicated as a cause of Alzheimer's disease. Synthesized as a membrane-bound protein, the neprilysin ectodomain is released into the extracellular domain after it has been transported from the Golgi apparatus to the cell surface.

Aggressive angiomyxoma

Angiomyxoma is a myxoid tumor involving the blood vessels.

Hemifacial hypertrophy human disease

Hemifacial hypertrophy abbreviated as (HFH) is rare congenital disease characterized by unilateral enlargement of the head and teeth. It is classified as true HFH (THFH) with unilateral enlargement of the viscerocranium, and partial HFH (PHFH) in which not all structures are enlarged. Hemifacial hypertrophy can cause a wide spectrum of defects or may involve only muscle or bone. it is usually treated surgically. It is believed to be a minor form of hemihypertrophy.

Epithelial-myoepithelial carcinoma Human disease

Epithelial-myoepithelial carcinoma (EMCa) is a rare malignant tumour that typically arises in a salivary gland and consists of both an epithelial and myoepithelial component. They are predominantly found in the parotid gland and represent approximately 1% of salivary gland tumours.

Hyalinizing clear cell carcinoma

Hyalinizing clear cell carcinoma (HCCC) is a rare malignant salivary gland tumour, with a good prognosis, that is usually found on the tongue or palate.

A sialoblastoma is a low-grade salivary gland neoplasm that recapitulates primitive salivary gland anlage. It has previously been referred to as congenital basal cell adenoma, embryoma, or basaloid adenocarcinoma. It is an extremely rare tumor, with less than 100 cases reported worldwide.

Rhabdomyoma is a benign mesenchymal tumor of skeletal muscle, separated into two major categories based on site: Cardiac and extracardiac. They are further separated by histology: fetal, juvenile (intermediate), and adult types. Genital types are recognized, but are often part of either the fetal or juvenile types. The fetal type is thought to recapitulate immature skeletal muscle at about week six to ten of gestational development.

Smooth muscle tumor of uncertain malignant potential, abbreviated STUMP, is an uncommon tumor of the uterine smooth muscle that may behave like a benign tumor or a cancerous tumor.

Plexiform angiomyxoid myofibroblastic tumor (PAMT), also called plexiform angiomyxoma, plexiform angiomyxoid tumor, or myxofibroma, is an extremely rare benign mesenchymal myxoid tumor along the gastrointestinal tract. Most of PAMTs occur in the gastric antral region, but they can be situated anywhere in the stomach. There is one recorded case of PAMT located in duodenum.

References

  1. 1 2 3 4 5 6 7 8 9 10 Smith BC, Ellis GL, Meis-Kindblom JM, Williams SB (May 1995). "Ectomesenchymal chondromyxoid tumor of the anterior tongue. Nineteen cases of a new clinicopathologic entity" (PDF). Am J Surg Pathol. 19 (5): 519–30. doi:10.1097/00000478-199505000-00003. PMID   7726361.
  2. Woo VL, Angiero F, Fantasia JE (May 2005). "Myoepithelioma of the tongue". Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 99 (5): 581–9. doi:10.1016/j.tripleo.2004.12.016. PMID   15829881.
  3. 1 2 Goveas N, Ethunandan M, Cowlishaw D, Flood TR (November 2006). "Ectomesenchymal chondromyxoid tumour of the tongue: Unlikely to originate from myoepithelial cells". Oral Oncol. 42 (10): 1026–8. doi:10.1016/j.oraloncology.2006.06.004. PMID   17011812.
  4. Seo SH, Shin DH, Kang HJ, Choi KU, Kim JY, Park do Y, Lee CH, Sol MY, Lee JC (October 2010). "Reticulated myxoid tumor of the tongue: 2 cases supporting an expanded clinical and immunophenotypic spectrum of ectomesenchymal chondromyxoid tumor of the tongue". Am J Dermatopathol. 32 (7): 660–4. doi:10.1097/DAD.0b013e3181d7d3bf. PMID   20661118.
  5. 1 2 3 4 5 Kannan R, Damm DD, White DK, Marsh W, Allen CM (October 1996). "Ectomesenchymal chondromyxoid tumor of the anterior tongue: a report of three cases". Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 82 (4): 417–22. PMID   8899780.
  6. 1 2 3 4 5 Pires FR, Abrahão AC, Cabral MG, Azevedo RS, Horta MC, Martins CR, de Almeida OP, Chen SY (December 2009). "Clinical, histological and immunohistochemical features of ectomesenchymal chondromyxoid tumor". Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 108 (6): 914–9. doi:10.1016/j.tripleo.2009.07.007. PMID   19800267.
  7. 1 2 3 Angiero F (November 2010). "Ectomesenchymal chondromyxoid tumour of the tongue. A review of histological and immunohistochemical features". Anticancer Res. 30 (11): 4685–9. PMID   21115924.
  8. Dickson BC, Antonescu CR, Argyris PP, et al. (Oct 2018). "Ectomesenchymal Chondromyxoid Tumor: A Neoplasm Characterized by Recurrent RREB1-MKL2 Fusions". Am J Surg Pathol. 42 (10): 1297–305. PMID   29912715.
  9. Nigam S, Dhingra KK, Gulati A (February 2006). "Ectomesenchymal chondromyxoid tumor of the hard palate--a case report". J Oral Pathol Med. 35 (2): 126–8. doi:10.1111/j.1600-0714.2006.00368.x. PMID   16430745.

Further reading