ODONTOGENIC MYXOMA

CASE PRESENTATION

NAME:SUDESH

AGE/SEX:28/F

CHIEF COMPLAINT: patient complains of hard swelling in upper left front gums region since 3 months.

HISTORY OF PRESENT ILLNESSpatient was apparently alright 3 months back.

During that time a small hard swelling appeared in upper left front gums region & has progressively increased to the present size.

H/O heaviness felt on left side of the nose.

No history of pain.

No history of sinus opening or discharge.

PAST MEDICAL HISTORY:no history of past systemic illness

DRUG HISTORY: no history of medication & drug allergy

PAST DENTAL HISTORY: there is no history of past dental treatment

FAMILY HISTORY: there is no relevant family history.

PERSONAL HISTORY: vegetarian by dietary habits & there is no other relevant personal history.

HISTORY OF HABITS:there is no history of habits.

GENERAL PHYSICAL EXAMINATION : patient is calm conscious, well built & well oriented with respect to time place & person .

All the vital signs of the patient are normal

EXTRAORAL EXAMINATION

•Dome shaped swelling •Left lower maxillary region•Extending anteroposteriorly from midline just below the nose till left nasolabial fold slightly raising the left ala & extending superoinferiorly from left ala till the line of lip closure.(1x1.5)cm in size.

Skin over the swelling appears normal in color & surface of the swelling appears smooth.

Temperature over the swelling is normal.

Swelling is bony hard & surface of the swelling appears smooth on palpation.

Maxillary sinus: no tenderness

Lymph nodes: non tender & not palpable.

TMJ: interincisal opening is normal, no deviation on opening, no clicking, no tenderness, no crepitation.

Hard tissue : Proclined upper anteriors. Angles class 2 molar relation.

No carious teeth.

No calculus, no pockets.

INTRAORAL EXAMINATION

Swelling present over buccal alveolus wrt 21,22,23

(1.5x1) cm in size.

Mucosa over the swelling appears normal in color & surface of the swelling appears smooth.

Swelling is bony hard & surface of the swelling appears smooth on palpation, with only a single groove like area palpable in the mid of the swelling extending superoinferiorly.

Associated teeth are vital on examination

PROVISIONAL DIAGNOSIS Angle’s class 2 div.1 malocclusion. UNICYSTIC AMELOBLASTOMA

DIFFERENTIAL DIAGNOSIS

CENTRAL GIANT CELL GRANULOMA CENTRAL HAEMANGIOMA

INVESTIGATIONS ADVISED

COMPLETE HAEMOGRAM, BLEEDING TIME, CLOTTING TIME ALKALINE PHOSPHATASE, CALCIUM BLOOD LEVELS ORTHOPENTOMOGRAM

CECT PNS & FACE

HISTOPATHOLOGICAL EXAMINATION

RADIOLOGICAL DIFFERENTIAL DIAGNOSIS

Central Ossifying fibroma Central giant cell granuloma

Central haemangioma Ameloblastoma

BLOOD INVESTIGATIONS: All blood counts are within normal limits.

MACROSCOPIC FINDINGS: Gritty bony tissue mixed with soft yellowish jelly like tissues felt with instrumentation during biopsy procedures.

FINAL DIAGNOSIS ODONTOGENIC MYXOMA

TREATMENT ENUCLEATION & CURRETAGE RESECTION

DISCUSSION

Myxoma is a benign, slow-growth, mesenchymal-stemed and locally aggressive neoplasia.

Virchow coined this term in 1863, because he believed that, as it happens with the umbilical chord, this disease had mucin.

Myxomas may involve hard and soft tissue (heart, subcutaneous, skin, and others).

Usually, when it involves bony tissue, it affects the facial bones (Allphin AL 1993, Zachariades N 1987, Wachter BG 2003).

The odontogenic myxoma is a rare, benign tumor that does not shed metastasis and involves the maxillo-mandibular complex.

When involving the maxilla, odontogenic myxomas can expand to inside the maxillary sinus, and are then diagnosed later only after having grown to larger sizes.

They may still involve the palate, orbit and nasal cavity, causing symptoms associated with these structures (Allphin AL 1993, Zachariades N 1987, Piatelli A 1994).

The odontogenic nature of the maxillary myxomas stemming from a tooth germ may be proven through the following facts: It rarely occurs in other bones if not on the face. It has a histological similarity with the dental mesenchyma. It is associated with unerupted or absent teeth.

There is a sporadic presence of epithelial islands or odontogenic tissue inside the myxomatous stroma. (Zachariades N 1984, Wachter BG 2003) .

•According to the latest WHO's classification of odontogenic tumors, in 1992, the myxoma is considered a tumor of the odontogenic mesenchyma, with or without the presence of odontogenic epithelium.

•4.72% of all odontogenic tumours (Kramer IRH, 1992).

EPIDEMIOLOGY

Odontogenic maxillary myxomas were first mentioned in the literature by Thoma and Goldman in 1947.

Usually affect adolescents and young adults, between the second and third decades of life.

very rarely affecting people before 10 years of age or after 50 years of age.

There is no consensus regarding location predilection (maxilla or mandible) and gender (males or females) and racial predilection (Allphin AL 1993, Costa ALL 1996) .

CLINICAL FEATURES

Like most odontogenic tumors, the odontogenic myxomas are asymptomatic, causing pain, paresthesia or asymmetries only when they take on larger sizes.

Slow growth, however locally aggressive. May cause divergence or root resorption, tooth shifting. (Zachariades N 1987, Piatelli A 1994, Costa ALL 1996) .

CLINICAL FEATURES

In a study affected individuals were within the age range of 10 and 39 years(33 cases), and they affect mostly the posterior portion of the mandible. (Simon ENM 2004).

Since most cases are advanced, the most common symptoms were pain and cortical bone perforation with invasion of soft tissue. (Simon ENM 2004).

RADIOGRAPHIC FEATURES

Most commonly multilocularity and root resorption (Simon ENM 2004).

Although the radiographic aspects are markedly variable, they are always radiolucent.

They may present with the aspect of soap bubbles, tennis racket and honey combs; there have been reports of a sun ray aspect  (Peltola J 1994, Wachter BG 2003).

In a retrospective radiographic analysis of 21 cases, the mandible was seen to be more involved than the maxilla (Peltola J, 1994).

Unilocular forms are usually located in the anterior maxilla, while the multilocular forms involve the posterior region (Peltola J, 1994).

Both conventional radiographs and CT scans must be used in the radiographic investigation in order to

• size the tumor, • define its margins, • establish bony septum aspects• and investigate presence of cortical

perforation (MacDonald-Jankowski DS 2004).

HISTOLOGICAL FEATURES

Abundant mucosal intercellular substance, made up of eosinophilic lax connective tissue,

Immersed in this stroma there are spindle-like cells and star-shaped cells with elongated cytoplasm, with or without small masses of inactive odontogenic epithelium.

Myxoma cases with characteristics that had not been described before in the literature, having presence of round calcified bodies made up of bony-cement tissue and islands of active odontogenic epithelium seen (Oygür T 2001, .Kimura A 2001).

DIFFERENTIAL DIAGNOSIS

Histologically, differential diagnosis:(Costa ALL, 1996). rabdomyosarcoma myxoid liposarcoma neurogenic sarcoma neurofibroma lipoma fibroma chondromyxoid and nodular faciitis.

Clinical and radiographic: Intraosseous hemangioma, Cherubism, Aneurysmatic bony cyst, Fibrous dysplasia, Ameloblastoma, Gigantic cells central lesion, Traumatic bony cyst Odontogenic cysts (radicular, lateral

periodontal, dentigerous and keratocyst) (Costa ALL 1996, Peltola J 1994).

DIFFERENTIAL DIAGNOSIS

TREATMENT

Since odontogenic myxomas bear a high risk of recurrence, mainly due to its gelatinous aspect and having no capsule, it is necessary that the initial treatment be very efficient.

Resection with broad margins is the most indicated treatment (Simon ENM, MacDonald-Jankowski DS, 2004) .

FOLLOW-UP

Post-operative observation, especially in the first two years, period of the greatest recurrence rate. (Piatelli A, 1994).