Connective Tissue Lesions PDF

CONNECTIVE TISSUE LESIONS Peripheral ossifying fibroma – a gingival mass in which islands of woven (immature) bone and...

CONNECTIVE TISSUE LESIONS Peripheral ossifying fibroma – a gingival mass in which islands of woven (immature) bone and osteoid are seen. – Surface is typically ulcerated Peripheral odontogenic fibroma – well-vascularized, nonencapsulated fibrous c/t. – Distinguishable feature: strands I. FIBROUS LESIONS of odontogenic epithelium – Usually, non-ulcerated REACTIVE HYPERPLASIAS – comprise a group of fibrous connective tissue lesions that commonly occur in oral mucosa as a result of injury – present as submucosal masses that may become secondarily ulcerated when traumatized such Giant cell fibroma as during mastication. – A focal fibrous Treatment: Surgical excision hyperplasia in A. Peripheral Fibroma which many of Clinical Features: which are ▪ is a reactive hyperplastic mass that occurs multinucleated, on the gingiva; derived from connective stellate shape. tissue of the submucosa or periodontal – One form of this lesion is known as ligament retrocuspid papilla of the mandible. ▪ may occur at any age Differential Diagnosis: ▪ more common in female ▪ Clinically, these lesions usually are not ▪ most affected is the gingiva anterior to the confused with anything else. However, permanent molars some overlap may be noted with pyogenic ▪ present clinically as either a stalked granuloma and, rarely, peripheral giant cell (pedunculated) or a broad-based (sessile) granuloma, when these two lesions do not mass have a prominent vascular component. Treatment: ▪ local excision B. Generalized Gingival Hyperplasia Etiology: ▪ Local factors: Plaque, calculus, bacteria ▪ Hormonal imbalance: estrogen, testosterone  ▪ Drugs: phenytoin(Dilantin), Cyclosporine, D. Denture-induced fibrous hyperplasia Nifedipine, other calcium channel blockers Etiology: ▪ Leukemia ▪ related to the ▪ Genetic factors/syndromes chronic trauma Clinical features: produced by an ▪ increase in bulk of the free and attached ill-fitting denture gingiva, especially the interdental papillae ▪ This lesion has also been referred to by ▪ stippling is lost several older synonyms, including ▪ gingival margins become rolled and inflammatory hyperplasia, denture blunted. hyperplasia, and epulis fissuratum. ▪ The consistency of the gingiva ranges from Clinical features: soft and spongy to firm and dense ▪ a common lesion that occurs in the ▪ A range of color from red-blue to lighter vestibular mucosa than surrounding tissue is also seen; ▪ less commonly along the mandibular Treatment: lingual sulcus ▪ Attentive oral hygiene – to minimize the ▪ painless folds of fibrous tissue surrounding effects of inflammation the overextended denture flange. ▪ Gingivoplasty or gingivectomy in Treatment: combination with- prophylaxis, oral ▪ Prolonged removal of denture hygiene instruction, comprehensive home ▪ Surgical excision care program ▪ Construction of new denture/ relining of C. Focal Fibrous the old one Hyperplasia Etiology: ▪ is a reactive NEOPLASMS lesion usually A. Solitary fibrous tumor caused by ▪ a benign proliferation of spindle cells of chronic trauma to oral mucous disputed but probable fibroblastic origin membranes. ▪ Oral counterpart of pleural solitary fibrous tumor Clinical Features: ▪ Buccal mucosa commonly affected ▪ is a reactive lesion usually caused by ▪ Circumscribed chronic trauma to oral mucous membranes. Treatment: ▪ is typically found in frequently traumatized ▪ Surgical excision areas, such as the buccal mucosa, the B. Myxoma lateral border of the tongue, and the lower Clinical Features: lip ▪ a soft tissue ▪ painless broad based swelling neoplasm ▪ Do not exceed 1-2 cm composed of Etiology: gelatinous ▪ Tongue: material that has a myxoid appearance - Neurofibroma histologically - Neurilemmoma ▪ Slow-growing - Granular cell tumor ▪ Asymptomatic sub-mucosal mass, usually ▪ Lower lip, Buccal mucosa: in the palate - Lipoma ▪ No gender predilection - Mucocele ▪ Occur at any stage - Salivary gland tumor Treatment: Treatment: ▪ Surgical excision ▪ Surgical excision ▪ require conservative therapy only B. Nasopharyngeal angiofibroma D. Myofibroblastic tumors Clinical features: Clinical features: ▪ also known as juvenile nasopharyngeal ▪ Myofibromatosis and myofibromas angiofibroma represent benign proliferations of ▪ This lesion characteristically produces a myofibroblasts mass in the nasopharynx, over time leads - Myofibromatosis – multifactorial and to obstruction or epistaxis occurs in infants ▪ Palatal expansion - Myofibroma – solitary and occurs over ▪ benign and slow-growing but a wide age range unencapsulated and locally invasive ▪ Cause: Unknown ▪ Symptom triad: recurrent epistaxis, nasal ▪ have a predilection for the head and neck, obstruction, and mass effect within the in particular, the oral cavity. nasopharynx. ▪ occur in soft tissues or in bone ▪ slow-growing, circumscribed mass Treatment: ▪ radiation, exogenous hormone Treatment: administration, sclerosant therapy, and ▪ Local excision embolization E. Fibromatosis C. Giant cell angiofibroma ▪ comprises a group of locally aggressive Clinical features: neoplasms that show infiltrative, ▪ rare soft tissue tumor that was first destructive, and recurrent growth but no described in the orbit tendency to metastasize. ▪ Extraorbital sites: submandibular region, Clinical features: parascapular area, posterior mediastinum ▪ Firm asymptomatic masses ▪ Occur in the oral cavity ▪ They are typically seen in children and ▪ Present as a slow growing nodule or mass young adults, with females affected twice with normal overlying mucosa as often as males. ▪ Benign and no tendency to metastasize ▪ Most common site: shoulder, trunk Treatment: ▪ Mandible and contiguous soft tissues are ▪ Surgical excision most often involved intraorally C. Nodular fasciitis ▪ slow-growing, circumscribed mass Clinical features: Treatment: ▪ also known as pseudosarcomatous ▪ Recurrence rate: 20-60% fasciitis ▪ Aggressive surgical approach is ▪ a well recognized entity representing a recommended fibrous c/t growth F. Fibrosarcoma ▪ Cause: Unknown ▪ defined as a rare malignant spindle cell ▪ Trauma is believed to be important in many tumor showing a herringbone or interlacing cases because of the location of lesions fascicular pattern and no expression of over bony prominences, such as: angle of other connective tissue cell markers. the mandible, zygoma ▪ Etiology: Unknown ▪ No gender predilection ▪ Young adults and adults are usually Clinical features: affected ▪ a rare soft tissue and bony malignancy of the head and neck Differential Diagnosis: ▪ Young adults are most commonly affected ▪ Fibromatosis ▪ This is an infiltrative neoplasm that is more ▪ Fibrous histiocytoma of a locally destructive problem than a ▪ Fibrosarcoma metastatic problem. Treatment: Treatment: ▪ Conservative surgical excision ▪ Wide surgical excision FIBROHISTIOCYTIC TUMORS II. VASCULAR LESIONS A. Benign fibrous histiocytoma REACTIVE LESIONS AND CONGENITAL Clinical features: LESIONS ▪ a.k.a dermatofibroma ▪ e fibroblastic neoplasms that rarely occur A. Lymphangioma in oral soft tissues, skin, or bone. ▪ Lesions of adults ▪ Painless and may be ulcerated ▪ Benign fibrous histiocytoma B. Malignant fibrous histiocytoma Clinical features: Etiology: ▪ an infrequently reported lesion in the head ▪ Congenital lesion and neck, most commonly made ▪ Appears within the first 2 decades of life diagnosed of soft tissue sarcoma in the Clinical features: rest of the body- in particular, lower leg ▪ Painless, nodular, vesicle-like swellings ▪ May also occur in bone ▪ On palpation it may produce crepitant ▪ Occurs in late adult like sound ▪ Rare in children ▪ Tongue is the most intraoral site ▪ Men are affected more ▪ Extremities and retroperitoneum are Treatment: favored sites ▪ Usually surgically removed ▪ Also been reported in the mandible and NEOPLASMS maxilla A. Hemangiopericytoma Treatment: ▪ a rare neoplasm that was originally ▪ Wide surgical excision described as a vascular tumor derived from the pericyte. ▪ believed to be a modified smooth muscle cell ▪ Appear as a mass in any location of the body across wide age spectrum ▪ Microscopically, the neoplasm is characterized by a proliferation of well- differentiated, oval to spindle-shaped mesenchymal cells separated by small, slit-like vascular channels. Treatment: ▪ Wide surgical excision B. Angiosarcoma ▪ a rare neoplasm of endothelial cell origin and unknown cause ▪ Scalp is the usual location ▪ Occasional lesion has been reported in the maxillary sinus and oral cavity ▪ consists of an unencapsulated proliferation of anaplastic endothelial cells enclosing irregular luminal spaces. ▪ has an aggressive clinical course ▪ poor prognosis. II. NEURAL LESIONS Differential Diagnosis: REACTIVE LESIONS AND CONGENITAL ▪ Neurofibroma LESIONS ▪ Schwannoma A. Traumatic Neuroma ▪ Palisaded encapsulated neuroma ▪ Salivary gland tumors Etiology: ▪ Lipoma ▪ Caused by injury to a peripheral nerve ▪ Other mesenchymal neoplasms ▪ trauma from a surgical procedure such as a ▪ Focal fibrous hyperplasia - is a common tooth extraction, from a local anesthetic reactive lesion that should be included in a injection, or from an accident differential diagnosis. Clinical features: Treatment: ▪ associated with pain Surgically excised and generally do not ▪ Radiating facial pain occasionally may be recur caused by a traumatic neuroma B. Schwannoma ▪ Seen in adults ▪ mental foramen is the most common Etiology: location, followed by extraction sites in the ▪ Also known as Neurilemmoma anterior maxilla and the posterior ▪ is a benign neoplasm that is derived from a mandible. proliferation of Schwann cells of the ▪ lower lip, tongue, buccal neurilemma, or nerve sheath. ▪ mucosa, and palate - common soft tissue ▪ As the lesion grows, the nerve is pushed locations aside and does not become enmeshed NEOPLASM within the tumor. A. Granular cell tumors Clinical features: Etiology: ▪ an encapsulated submucosal mass that ▪ formerly known as granular cell presents typically as an asymptomatic myoblastoma, is an uncommon benign lump in patients of any age tumor of unknown cause ▪ tongue is the favored location ▪ Origins from skeletal muscle, ▪ Bony lesions produce a well-defined macrophages, undifferentiated radiolucent pattern with a corticated mesenchymal cells, and pericytes have periphery and may cause pain or been suggested but are unproven paresthesia. ▪ A related lesion known as congenital ▪ Usually slow growing but may undergo a gingival granular tumor (congenital epulis) sudden increase in size. is composed of cells that are light microscopically identical to those of GCT. Treatment: ▪ Surgically excised and generally do not Clinical features: recur ▪ Seen in children to elderly ▪ Prognosis is excellent ▪ In the head and neck, the tongue is by far the most common location for granular cell tumors (any oral location may be affected) ▪ Uninflamed asymptomatic mass less than 2 cm in diameter C. Neurofibroma ▪ is caused by a mutation in the RET oncogene resulting in a single amino acid substitution of a single methionine to threonine that affects a critical region of the tyrosine kinase catalytic core Clinical features: Etiology: ▪ consists of medullary carcinoma of the ▪ appear as solitary lesions or as multiple thyroid, pheochromocytoma of the adrenal lesions as part of the syndrome gland and neurofibromas of the skin neurofibromatosis (Von Recklinghausen’s ▪ appear early in life as small, discrete disease of skin) nodules on the conjunctiva, labia, or ▪ Cause: Unknown larynx, or in the oral cavity. ▪ Inherited as an autosomal-dominant traits ▪ The oral lesions are seen on the tongue, Clinical features: lips, and buccal mucosa ▪ Uninflamed asymptomatic, submucosal Treatment: mass. ▪ Surgical excision and have little chance to ▪ commonly affected areas: tongue, buccal recurrence mucosa, and vestibule ▪ Early detection of mucosal neuromas ▪ Lesion may be so numerous and prominent therefore is of utmost importance in that they become cosmetically significant establishing the diagnosis or calling ▪ Other important diagnosis signs: Axillary attention to other components of the Freckling (Crowe’s sign) and Iris freckling syndrome (Lisch spots) E. ▪ Flaring of IAN (blunderbuss foramen) Palisaded Encapsulated Neuroma Differential diagnosis: (Solitary Circumscribed Neuroma) ▪ Traumatic fibroma ▪ Granular cell tumor ▪ Lipoma ▪ ** a diffuse neurofibroma resulting in macroglossia may require differentiation from lymphangioma and possibly amyloidosis. Etiology: Treatment: ▪ Neural origin ▪ Surgical excision and have little chance to ▪ not associated with neurofibromatosis or recurrence MEN III. ▪ Prognosis: Px who has had ▪ typically in the palate and occasionally on neurosarcomatous change of a pre-existing the lips lesion is poor ▪ This domes-haped nodule is encapsulated D. and exhibits a fascicular microscopic Mucosal Neuroma of pattern with some suggestion of nuclear Multiple Endocrine palisading Neoplasia Syndrome Type III Treatment: ▪ Surgically removal, recurrence is unexpected Etiology: ▪ Is inherited as an autosomal-dominant trait ▪ The clinical stigma of this syndrome are related to a defect in neuroectodermal tissue F. Malignant Peripheral III. MUSCLE LESIONS Nerve Sheath Tumor REACTIVE LESIONS Etiology: ▪ rare malignancy A. Myositis Ossificans that may Etiology: develop from a ▪ an uncommon reactive lesion of skeletal preexisting muscle. neurofibroma or ▪ It may appear in the muscles of the head de novo. and neck. ▪ Origin: believed to be a Schwann cells and ▪ intramuscular inflammatory process in possibly other nerve sheath cells which ossification occurs Clinical features: ▪ Expansible mass; usually asymptomatic Clinical features: ▪ In bone, where it is believed to arise most may be seen in either of two forms: often from the inferior alveolar nerve, it ▪ progressive systemic disease (myositis presents as a dilation of the mandibular ossificans progressiva) of unknown cause canal or as a diffuse radiolucency. ▪ or as a focal single-muscle disorder ▪ Pain or paresthesia may accompany the traumatic myositis ossificans) lesion in bone ▪ Most common affected: masseter and sternocleidomastoid Treatment: ▪ Wide surgical excision. Recurrence is Treatment: common and metastases are frequently ▪ Surgical excision and have a little chance seen of recurrence ▪ Prognosis: varies from fair to good, depending on clinical circumstances NEOPLASMS G. Olfactory Neuroblastoma A. Leiomyoma and Leiomyosarcoma Etiology: ▪ Leiomyoma - most commonly arise in the ▪ also known as esthesioneuroblastoma, muscularis layer of the gut and in the body ▪ is a rare malignant lesion that arises from of the uterus olfactory tissue in the superior portion of ▪ appear as slow-growing, asymptomatic the nasal cavity submucosal masses, usually in the tongue, ▪ Occurring in young adults hard palate, or buccal mucosa. ▪ may result in epistaxis, rhinorrhea, or nasal ▪ They may be seen at any age; 1 to 2 cm in obstruction, or it may present as polyps in diameter the roof of the nasal cavity. ▪ spindle cell proliferation shares many ▪ also result in a nasopharyngeal mass or an similarities with neurofibroma, invasive maxillary sinus lesion. schwannoma, fibromatosis, and myofibroma Microscopically differential diagnosis: ▪ Surgical excision and recurrence is ▪ Lymphoma unexpected ▪ Embryonal rhabdomyosarcoma, Ewing’s sarcoma, and undifferentiated carcinoma ▪ Leiomyosarcoma – arises in the Treatment: retroperitoneum, mesentery, omentum, or ▪ Surgery and radiation; recurrence is subcutaneous and deep tissue of the limbs uncommon ▪ Reported in all age groups ▪ Metastasis, usually to local nodes or lungs ▪ Microscopic diagnosis is a considerable occurs infrequently challenge because of similarities to other spindle cell sarcomas ▪ wide surgical excision; Metastasis to lymph nodes or lung is not uncommon  IV. FAT LESIONS B. Rhabdomyoma A. Lipoma Etiology: ▪ rare lesions ▪ have a predilection for the soft tissues of the head and neck. ▪ The oral sites: floor of the mouth, soft palate, tongue, and buccal mucosa. ▪ mean age = 50 years, and the age range Etiology: extends from children to older adults ▪ uncommon neoplasms that may occur in ▪ Asymptomatic any region of the oral cavity. ▪ Well-defined submucosal mass ▪ The buccal mucosa, tongue, and floor of the mouth are among the more common Treatment: locations. ▪ Surgically excision and recurrence is Clinical features: unexpected ▪ Asymptomatic, yellowish, submucosal mass C. Rhabdomyosarcoma ▪ The overlying epithelium is intact, and Etiology: superficial blood vessels are usually Divided into 3 microscopic forms: evident over the tumor ▪ Embryonal – consists of primitive round B. Liposarcoma cells in which striations are rarely found ▪ Alveolar – composed of round cells but in the compartmentalized pattern ▪ Pleomorphic – most well differentiated, contains strap or spindle cells that often exhibit cross-striations Etiology: ▪ rarely encountered in soft tissues of the Treatment: head and neck. Combination of surgery, radiaton and ▪ It is a lesion of adulthood and may chemotherapy potentially occur at any site. ▪ It usually develops slowly and may be mistaken for a benign process. Treatment: ▪ Surgery or radiation ▪ Prognosis: fair to good