MESENCHYMAL (SOFT TISSUE) TUMORS OF OROFACIAL REGION

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MESENCHYMAL (SOFT TISSUE) TUMORS OF OROFACIAL REGION by Mind Map: MESENCHYMAL (SOFT TISSUE) TUMORS OF OROFACIAL REGION

1. FIBROBLASTS

1.1. Benign

1.1.1. Fibroma

1.1.1.1. Introduction

1.1.1.1.1. One of the commonest tumors of oral cavity.

1.1.1.1.2. Not a true neoplasm in most cases.

1.1.1.1.3. Reactive hyperplasia of fibrous CT in response to local trauma or irritation.

1.1.1.2. C/F

1.1.1.2.1. Age incidence - 4th to 6th decades.

1.1.1.2.2. Sex incidence - 1:2, male to female ratio.

1.1.1.2.3. Site

1.1.1.2.4. Sign & symptoms -

1.1.1.2.5. D.D

1.1.1.3. H/P

1.1.1.3.1. Dense mass of fibro- cellular CT covered by a thin, atrophic stratified squamous epithelium.

1.1.1.3.2. CT - densely collagenized - collagen bundles arranged in a radiating, circular or haphazard fashion.

1.1.2. Giant cell fibroma

1.1.2.1. Introduction

1.1.2.1.1. it is a fibrous tumor.

1.1.2.1.2. Probably a true neoplasm with distinct h/p features

1.1.2.2. C/F

1.1.2.2.1. Age incidence – First 3 decades of life

1.1.2.2.2. Sex incidence – Slight female

1.1.2.2.3. Site

1.1.2.2.4. Signs & symptoms -

1.1.2.2.5. RETROCUSPID PAPILLA

1.1.2.2.6. D.D

1.1.2.3. H/P

1.1.2.3.1. Mass of loose, fibrous CT covered by relatively hyperplastic surface epithelium.

1.1.2.3.2. CT mass contains numerous plump, spindle / stellate shaped fibroblasts, many of which are multinucleated.

1.1.3. INFLAMMATORY FIBROUS HYPERPLASIA (Epulis Fissuratum)

1.1.3.1. Introduction

1.1.3.1.1. Misnomer

1.1.3.2. Etiology

1.1.3.2.1. Flanges of ill fitting dentures

1.1.3.2.2. poor oral hyegine

1.1.3.3. C/F

1.1.3.3.1. Age incidence – Middle aged to elderly persons.

1.1.3.3.2. Sex incidence – More in females.

1.1.3.3.3. Site

1.1.3.3.4. Signs and symptoms -

1.1.3.3.5. LEAFLIKE DENTURE FIBROMA

1.1.3.4. H/P

1.1.3.4.1. Hyperplastic fibrous CT, covered by a hyperplastic stratified squamous epithelium which shows numerous papillary folds.

1.1.3.4.2. Occasionally due to chronic irritation, osseous or chondromatous metaplasia is seen resulting in bone / cartilage formation within the lesion.

1.1.4. Inflammatory papillary hyperplasia (DENTURE PAPILLOMATOSIS)

1.1.4.1. Introduction

1.1.4.1.1. Reactive tissue growth that usually but not always occurs beneath a denture.

1.1.4.1.2. certain factors appear to be related

1.1.4.2. C/F

1.1.4.2.1. Age incidence – Elderly persons

1.1.4.2.2. Site

1.1.4.2.3. Signs & symptoms -

1.1.4.3. H/P

1.1.4.3.1. CT

1.1.4.3.2. covered by hyperplastic stratified squamous epithelium

1.1.4.3.3. Both inflammatory fibrous and papillary hyperplasia sometimes demonstrate->

1.1.4.3.4. Proliferation of overlying epithelium

1.1.4.3.5. SHOULD NOT diagnose this as squamous cell carcinoma because epithelial cells are benign.

1.1.5. Fibrous histiocytoma

1.1.5.1. Introduction

1.1.5.1.1. Diverse group of tumors exhibiting both fibroblastic and histiocytic properties.

1.1.5.1.2. True neoplasm.

1.1.5.1.3. arise from tissue histiocyte which later assumes fibroblastic properties.

1.1.5.2. C/F

1.1.5.2.1. Age incidence – Middle to older aged.

1.1.5.2.2. Site of occurrence – Oral tumors are rare. Occurs in buccal mucosa & vestibule.

1.1.5.2.3. Signs & symptoms –

1.1.5.3. D.D

1.1.5.3.1. FIBROMA

1.1.5.3.2. GIANT CELL FIBROMA

1.1.5.3.3. NEURILEMMOMA

1.1.5.3.4. NEUROFIBROMA

1.1.5.4. H/P

1.1.5.4.1. Proliferation of spindle shaped cells with vesicular nuclei.

1.1.5.4.2. Tumor cells arranged in short interlacing bundles (STORIFORM PATTERN).

1.1.6. Fibromatosis

1.1.7. Myofibroma

1.1.8. Pyogenic granuloma

1.1.8.1. Introduction

1.1.8.1.1. Non neoplastic, tumor like growth of oral cavity.

1.1.8.1.2. Originally believed to be caused by pyogenic organisms.

1.1.8.1.3. occur as a response to local trauma / irritation.

1.1.8.1.4. NOT a true granuloma as name suggests.

1.1.8.2. C/F

1.1.8.2.1. Age incidence – Children and young adults

1.1.8.2.2. Sex incidence – More in females.

1.1.8.2.3. Site

1.1.8.2.4. Signs & symptoms –

1.1.8.2.5. PREGNANCY TUMOUR

1.1.8.3. D.D

1.1.8.3.1. If PG occurs on gingiva, it must be differentiated from other epulis.

1.1.8.4. H/P

1.1.8.4.1. Resembles granulation tissue

1.1.8.4.2. Newly formed endothelium lined vascular channels.

1.1.8.4.3. Mixed inflammatory infiltrate of neutrophils and plasma cells.

1.1.9. Peripheral Giant Cell Granuloma

1.1.9.1. Introduction

1.1.9.1.1. Relatively common tumor like lesion of oral cavity.

1.1.9.1.2. Non neoplastic in nature, occurring as reaction to local trauma / irritation.

1.1.9.1.3. Giant cells within lesion believed to originate from either

1.1.9.1.4. Believed to be a soft tissue counterpart of Central Giant Cell granuloma.

1.1.9.2. C/F

1.1.9.2.1. Age incidence – 5th & 6th decades.

1.1.9.2.2. Sex incidence – 60% cases occur in females.

1.1.9.2.3. Site of occurrence – Exclusively on gingiva and edentulous alveolar ridge.

1.1.9.2.4. SIGNS & SYMPTOMS -

1.1.9.3. D.D

1.1.9.3.1. PERIPHERAL OSSIFYING FIBROMA

1.1.9.3.2. PYOGENIC GRANULOMA

1.1.9.3.3. FIBROMA

1.1.9.4. H/P

1.1.9.4.1. Proliferation of multinucleated giant cells within a background of plump spindle / ovoid mesenchymal cells and abundant hemorrhage.

1.1.9.4.2. Multinucleated giant cells within a background of mesenchymal cells and scattered hemorrhage seen in this picture.

1.1.10. Peripheral Ossifying Fibroma

1.1.10.1. Introduction

1.1.10.1.1. Relatively common reactive gingival growth

1.1.10.1.2. believed to develop initially as pyogenic granulomas =>fibrous maturation and subsequent calcification.

1.1.10.1.3. Mineralized product probably derived from PDL or periosteum

1.1.10.2. C/F

1.1.10.2.1. Age incidence – 1st and 2nd decades

1.1.10.2.2. Sex incidence – 2/3rd of cases occur in females

1.1.10.2.3. Site

1.1.10.2.4. Signs & symptoms -

1.1.10.3. D,D

1.1.10.3.1. All gingival swellings (epulis) must be considered in the DD

1.1.10.4. H/P

1.1.10.4.1. Fibrous proliferation associated with the formation of a mineralized product.

1.1.10.4.2. Variable mineralized component.

1.1.10.4.3. May consist of bone, cementum or even dystrophic calcification.

1.2. Malignant

1.2.1. Fibrosarcoma

1.2.1.1. C/F

1.2.1.1.1. Age incidence – Children & young adults

1.2.1.1.2. Site of occurrence – Most common in extremities. Can occur anywhere in head & neck.

1.2.1.1.3. Signs & symptoms:

1.2.1.2. D.D

1.2.1.2.1. Fibroma

1.2.1.2.2. Neurilemmoma

1.2.1.2.3. Neurofibroma

1.2.1.2.4. Fibrous histiocytoma

1.2.1.2.5. Malignant Fibrous histiocytoma

1.2.1.3. H/P

1.2.1.3.1. Well differentiated fibro sarcomas consist of fascicles of spindle shaped cells in a streaking pattern called “HERRING BONE PATTERN”.

1.2.1.3.2. Poorly differentiated tumors - cells are less organized, mild pleomorphism along with more mitotic activity noted.

1.2.1.3.3. produce less collagen than well differentiated tumors

1.2.2. Malignant fibrous histiocytoma

1.2.2.1. Introduction

1.2.2.1.1. Considered to be the most common soft tissue sarcoma in adults.

1.2.2.1.2. arise from malignant cells which show both fibroblastic as well as histiocytic features.

1.2.2.2. C/F

1.2.2.2.1. Age incidence – Older age group

1.2.2.2.2. Site of occurrence – Retro peritoneum and extremities. Rare in head and neck region.

1.2.2.2.3. Signs & symptoms – Rapidly expanding mass which may or may not be painful or ulcerated.

1.2.2.3. D.D

1.2.2.3.1. Fibroma

1.2.2.3.2. Fibrosarcoma

1.2.2.3.3. Fibrous histiocytoma

1.2.2.3.4. Neurilemmoma

1.2.2.3.5. Neurofibroma

1.2.2.4. H/P

1.2.2.4.1. Amongst the various subtypes, storiform- pleomorphic pattern is most common.

1.2.2.4.2. Short fascicles of spindle cells admixed with areas of pleomorphic giant cells.

2. VASCULAR

2.1. Benign

2.1.1. Hemangioma

2.1.1.1. Introduction

2.1.1.1.1. Earlier, Hemangiomas & developmental vascular malformations were clubbed together.

2.1.1.1.2. Now, Hemangiomas are considered hamartomas (developmental tumors)

2.1.1.2. C/F

2.1.1.2.1. Age incidence – Appear by 1st year

2.1.1.2.2. Sex incidence – 3:1 Female

2.1.1.2.3. Site

2.1.1.2.4. Signs & symptoms -

2.1.1.3. COMPLICATIONS

2.1.1.3.1. Complications seen in about 20% cases.

2.1.1.3.2. Ulceration, with or without secondary infection most common.

2.1.1.3.3. Hemorrhage also important, although significant blood loss may not occur.

2.1.1.3.4. Periocular hemangiomas can cause amblyopia, astigmatism etc.

2.1.1.3.5. Neck / laryngeal tumors may cause airway obstruction.

2.1.1.4. H/P

2.1.1.4.1. Depending upon size of vessels proliferating within the lesion

2.1.1.4.2. Hemangiomas are classified as

2.1.2. Vascular Malformations

2.1.2.1. Introduction

2.1.2.1.1. Classified according to type of vessel involved (capillary, venous or arterial) and also according to hemodynamics (high flow and low flow)

2.1.2.1.2. Port wine stain is an example of capillary malformation.

2.1.2.1.3. Differences between hemangioma & vascular malformations: -

2.1.3. Sturge-Weber Angiomatosis

2.1.4. Nasopharyngeal Angiofibroma

2.1.5. Hemangiopericy toma

2.1.6. Lymphangioma

2.1.6.1. Introduction

2.1.6.1.1. Benign hamartomatous tumors of lymphatic vessels.

2.1.6.1.2. Probably represent developmental malformations which arise as a result of sequestration of lymph vessels that do not communicate with rest of lymphatic system.

2.1.6.2. CLASSIFICATION: -

2.1.6.2.1. 1) LYMPHANGIOMA SIMPLEX (Capillary lymphangioma)

2.1.6.2.2. 2) CAVERNOUS LYMPHANGIOMA

2.1.6.2.3. 3) CYSTIC LYMPHANGIOMA (Cystic hygroma)

2.1.6.3. C/F

2.1.6.3.1. Age incidence – 50% lesions seen at birth, 90% develop by 2 years.

2.1.6.3.2. Site

2.1.6.3.3. Signs & symptoms -

2.1.6.4. D.D

2.1.6.4.1. HERPES SIMPLEX INFECTION

2.1.6.4.2. PEMPHIGUS

2.1.6.4.3. HERPES ZOSTER

2.1.6.4.4. CHICKEN POX

2.1.6.5. H/P

2.1.6.5.1. Typically composed of dilated lymph vessels in a loose fibro vascular connective tissue.

2.1.6.5.2. Vessels lined usually by single layer of flattened endothelial cells and contain lymph within the dilated spaces.

2.2. Malignant

2.2.1. Angiosarcoma

2.2.2. Kaposi Sarcoma

2.2.2.1. Introduction

2.2.2.1.1. occurrence in association with AIDS.

2.2.2.1.2. Current research indicates, it is caused by human herpes virus 8.

2.2.2.1.3. Most probably arises from endothelial cells

2.2.2.2. C/F

2.2.2.2.1. Classifications

2.2.2.3. H/P

2.2.2.3.1. 1)Patch stage –

2.2.2.3.2. 2) Plaque stage

2.2.2.3.3. 3) Nodular stage -

3. NERVES TISSUE

3.1. Benign

3.1.1. Traumatic Neuroma

3.1.1.1. Introduction

3.1.1.1.1. Reactive proliferation of neural tissue after transection or other damage to a nerve bundle.

3.1.1.1.2. After damage

3.1.1.2. C/F

3.1.1.2.1. Age incidence – Middle aged adults.

3.1.1.2.2. Sex incidence – Slightly more in female

3.1.1.2.3. Site of occurrence – Mental foramen area, tongue & lower lip.

3.1.1.2.4. Signs & symptoms -

3.1.1.3. H/P

3.1.1.3.1. Lesion is composed of interlacing proliferation of mature nerve bundles within a fibrous connective tissue stroma.

3.1.1.3.2. High power view shows cross sectioned nerve bundles within dense fibrous connective tissue.

3.1.2. Palisaded Encapsulated Neuroma

3.1.2.1. Introduction

3.1.2.1.1. Preferred term is SOLITARY CIRCUMSCRIBED NEUROMA

3.1.2.1.2. A common superficial nerve tumor of head & neck region.

3.1.2.1.3. Not a true neoplasm, but a reactive lesion, probably in response to trauma.

3.1.2.1.4. Not a true neoplasm, but a reactive lesion, probably in response to trauma.

3.1.2.2. C/F

3.1.2.2.1. Age incidence – 5th to 7th decades

3.1.2.2.2. Site

3.1.2.2.3. Signs & symptoms -

3.1.2.3. D,D

3.1.2.3.1. Neurofibroma

3.1.2.3.2. Neurilemmoma

3.1.2.3.3. Traumatic neuroma

3.1.2.3.4. Fibroma

3.1.2.3.5. Minor salivary gland neoplasms

3.1.2.4. H/P

3.1.2.4.1. This lesion appears as a completely / partially encapsulated mass of spindle cells arranged in interlacing fascicles.

3.1.2.4.2. Nuclear palisading may be seen in this lesion too, thereby resembling a schwannoma, but the characteristic verocay bodies are lacking.

3.1.3. NEURILEMMOMA (Schwannoma)

3.1.3.1. Introduction

3.1.3.1.1. It is a true neoplasm arising from Schwann cells.

3.1.3.1.2. 25% – 50% of all neurilemmoma occur in the head and neck region.

3.1.3.2. C/F

3.1.3.2.1. Age incidence – Young & middle aged.

3.1.3.2.2. Site

3.1.3.2.3. Signs & symptoms -

3.1.3.3. D,D

3.1.3.3.1. Neurofibroma

3.1.3.3.2. Solitary circumscribed neuroma

3.1.3.3.3. Fibroma

3.1.3.3.4. Minor salivary gland neoplasms

3.1.3.3.5. Fibrous histiocytoma

3.1.3.4. H/P

3.1.3.4.1. Usually encapsulated tumor showing 2 microscopic patterns in varying proportions – Antoni A & Antoni B.

3.1.3.4.2. Antoni A shows schwann cells palisading around central, acellular eosinophilic mass (Verocay body)

3.1.3.4.3. Antoni B shows haphazardly, randomly arranged Schwann cells within a loose myxomatous stroma.

3.1.3.4.4. In older neurilemmomas, degenerative changes like hemosiderin deposits, fibrosis, inflammation, hemorrhage etc can be seen (Ancient neurilemmoma)

3.1.4. Neurofibroma

3.1.4.1. Introduction

3.1.4.1.1. Most common neoplasm of peripheral nerves.

3.1.4.1.2. Arises from a mixture cells like schwann cells, perineural fibroblasts etc.

3.1.4.2. C/F

3.1.4.2.1. Age incidence – Young adults

3.1.4.2.2. Site

3.1.4.2.3. SIGNS & SYMPTOMS -

3.1.4.3. D.D

3.1.4.3.1. Neurilemmoma

3.1.4.3.2. Solitary circumscribed neuroma

3.1.4.3.3. Fibroma

3.1.4.3.4. Fibrous histiocytoma

3.1.4.3.5. Minor salivary gland neoplasms.

3.1.4.4. H/P

3.1.4.4.1. Presents as haphazardly arranged delicate spindle cells with wavy nuclei within a delicate, loose connective tissue containing delicate collagen fibrils.

3.1.4.4.2. Mast cells are seen in large numbers and are a diagnostic aid.

3.1.4.5. PLEXIFORM NEUROFIBROMA

3.1.4.5.1. Variant of Neurofibroma and is pathognomonic of Neurofibromatosis.

3.1.4.5.2. They consist of haphazardly organized mixtures of nerve fibrils.

3.1.4.5.3. Compact bundles of spindle cells proliferating into surrounding tissue can be seen.

3.1.5. Neurofibromatosis

3.1.5.1. Introduction

3.1.5.1.1. known as Von Recklinghausen’s disease of skin.

3.1.5.1.2. Relatively common hereditary condition.

3.1.5.1.3. Inherited as an autosomal dominant trait

3.1.5.2. Diagnostic criteria for neurofibromatosis type I

3.1.5.3. C/F

3.1.5.3.1. Signs & symptoms: -

3.1.5.3.2. few other notable c/f are :

3.1.5.3.3. ORAL MANIFESTATIONS: -

3.1.6. Multiple Endocrine Neoplasia Type 2B

3.1.7. Melanotic Neuroectodermal Tumor of Infancy

3.1.8. Paraganglioma

3.1.9. Granular Cell Tumor

3.1.10. Congenital Epulis

3.2. Malignant

3.2.1. Malignant peripheral nerve sheath tumor

3.2.1.1. Introduction

3.2.1.1.1. called as neurofibrosarcoma, neurogenic sarcoma or malignant schwannoma.

3.2.1.1.2. Almost half of the cases arise in patients with Neurofibromatosis.

3.2.1.2. C/F

3.2.1.2.1. Age incidence – Young adults.

3.2.1.2.2. Site

3.2.1.2.3. Signs & symptoms –

3.2.1.3. D.D

3.2.1.3.1. Fibrosarcoma

3.2.1.3.2. Fibroma

3.2.1.3.3. Fibrous histiocytoma

3.2.1.3.4. Minor salivary gland neoplasms

3.2.1.3.5. Central giant cell granuloma (in case of intrabony lesions only)

3.2.1.4. H/P

3.2.1.4.1. One of the most difficult neoplasms to diagnose histologically.

3.2.1.4.2. Tissue proliferation resembles a fibrosarcoma, but here spindle cells have a wavy nucleus.

3.2.1.4.3. Neurites can be seen within the lesion which can be of some help.

4. Classifications