2015/09/29

報告者： Intern Group B 指導醫師：陳玉昆 主任 林立民 醫師

及口腔病理科全體醫師

2015/09/29

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 General data: 陳暐翔

 X-ray finding: 邱仲凡

 Discussion: 廖信昱

 醫學倫理：許永潾

 PPT製作：全體組員

 報告：全體組員

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 Name: 陳X

 Sex: Male

 Age: 19 y/o

 Native: 高雄市

 Marital status: single

 Attending staff: OOO醫師

 First visit : 94/11/22

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 Came with his mother, 12 months s/p enucleation of the large cyst, came with his mother for follow-up of his multiple lesions.

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 This is a 19 year-old male who first visited our out-patient-department in 94/11/22 and was found multiple radiolucences over bimaxillary and arranged operation (biopsy &

decompression), and histopathologic report showed keratocystic odontogenic tumor over right mandible; left maxillary & mandible.

Then after a period of follow up, the patient

came back in 95/02/07 for extraction tooth 83 residual root at Pediatric Dentistry Department

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 Came back in 95/02/21 for enlargment of right mandible cyst orifice (tooth 83) and obturator fabrication, came back in 95/03/14 to extract tooth 84 for enlarging right mandible fistula, and in 95/05/16 for re-shaping the

decompression button (tooth 84), then in

95/07/18 for removing decompression button over left mandibular angle area, and in

95/11/21 for removing right obturator.

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 Then in 97/01/31, 38 area cystic lesion

persisted, we did extraction tooth 53 & 75 as decompression in 97/07/24, and 97/08/14 we found right maxilla keratocystic odontogenic tumor, left mandible odontogenic cyst and tooth 38 area dentigerous cyst. In 97/01/23, the patient got 13 impaction and consult

orthodontic department, the doctor of

orthodontic department suggested orthodontic treatment right now or until 31 eruption

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 In 98/07/14, patient was diagnosed of keratocystic odontogenic tumor over left posterior mandible, and we placed

decompression button and biopsy. In 99/07/03, the patient had increased size of tooth 38

dentigerous cyst and a biopsy & decompression button was set, also the histopathologic report:

infected odontogenic cyst.

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 Then, in 99/02/01 we did the keratocystic odontogenic tumor encleation over tooth 42, 43 area, and in 99/07/06, we found new cyst of tooth 23 area but only followed up at OPD. In 100/08/09, we found tooth 38, 48 swelling and 47 pushed posterior and we did decompression

& biopsy histopathologic report: inflammatory cell infiltration, then in 100/10/25, we found a new cyst over tooth 18 and kept following up.

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 In 101/08/27, two compression buttons were observed at left maxilla and cystic lesion at right maxilla so we arranged sedation anesthesia at

dental out-patient-department for special need for biopsy and place decompression button in 101/09/27. The operation was at sleeping mode but cough reflex severe, so we placed

compression button, penrose and set bacterial culture, also arranged operation (multiple

keratocystic odontogenic tumor over

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 Maxilla & mandible with impaction s/p Debulky with tooth 17, 18 removed +

decompression over right maxilla and cyst

enucleation with tooth 27, 28, 38, 47, and 48 removed over left maxilla + mandible in

101/10/26. And histopathologic report was hyperparakeratosis of tooth 46 distal, and keratocystic odontogenic tumor of bilateral mandible and maxilla.

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 This time after the patient has been followed up at out-patient-department for 4 months, we kept monitoring the operation outcome of

enucleation of the large cyst over right maxilla.

In 102/01/26, restoration of tooth 16 drop, the local-dental-clinic suggested extraction of tooth 16, so the extraction was performed in

102/04/11.

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 In 102/05/02, we arranged operation in

102/07/03, and the post-operative pathology report: keratocystic odontogenic tumor over

right and right posterior medial pterygoid plate, keratocystic odontogenic tumor over right

infraorbital and posterior, fibrotic tissue with multiple odontogenic epithelial rest over right mandible.

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 In 102/12/12, we arrange CT, check BUN, Cr for him, and CT report: 1) Shrinkage yet

persistence of odontogenic cyst in the right

aspect of the maxilla status post drainage tube insertion. 2) Absence of odontogenic cyst in the left aspect of the maxilla. 3) Mild bilateral

ethmoid and left maxillary sinusitis. Non- specific small lymph nodes (<1cm) in the

submental, the bilateral submandibular, jugulo- digastric, and the posterior cervical spaces

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 In 103/02/13, we arrange operation in 103/03/12 for him.

 Pt was suspected with Gorlin syndrome (Basal cell nevus syndrome).Pt denied the history of drug allgery. Pt denied the oral habits of

cigarette smoking, betel quid chewing and

alcohol drinking. 病人表示打完造影劑之後

會有過敏的反應 病人表示他的頭頂上有長

了一顆新的痣 高雄榮總皮膚科意見為觀察 No evidence of active progression ₁₅

 Past medical history

 Underlying disease: Denied

 Hospitalization (+), odontogenic cyst

 Surgery under GA (+), odontogenic cyst

 Allergy: contrast medium (Ultravist)

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 Past Dental History

 General routine dental treatment

 Attitude to dental treatment : co-operative

 Risk factors related to malignancy

 Alcohol (-)

 Betel quid (-)

 Cigarette (-)

 Special oral habits: Denied

 Irritation: Denied

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 2015/9/10

 Mandible:

There are two well-defined unilocular round shaped radiolucence with a corticated margin lesions. One is at right posterior region of mandible body and with bone expanding extending to cortical margin, measuring in about 3x3 cm in size. Another one is at right mandible ramus region and extends from retromolar pad region to mandibular canal region, measuring in about 2x1 cm in diameter.

 Maxilla:

There is a well-defined unilocular round shaped

radiolucece without a corticated margin over maxilla

extending from distal side of lateral incisor to mesial side of canine and from ridge between tooth 13 and 12 up to right maxilla sinus, measuring about 1x3 cm in size.

 Dental findings:

1. Missing tooth: 16, 17, 18, 27, 28, 38, 46, 47, 48 2. Restoration: tooth 36 (MO)

3. Tooth is mesial tilting

 2005/12/13

 2006/9/19

 2006/11/21

 2007/2/13

 2007/5/31

 2008/1/31

 2008/7/24

 2009/1/20

 2009/7/3

 2009/8/18

 2010/1/19

 2010/4/24

 2010/8/17

 2011/1/25

 2011/8/2

 2011/11/25

 2012/1/17

 2012/8/27

 2012/10/27

 2012/10/27

 2012/12/6

 2013/1/17

 2013/7/4

 2013/10/3

 2014/2/6

 2014/3/13

 2014/5/15

 2015/2/12

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Mandible:

 Bone expansion at buccal-lingual and up-down direction.

 The large one compress right mandibular canal

 The smaller one is fused with mandibular canal

Maxilla:

 The lesion invades in right nasal cavity.

 The lesion invades the root of tooth 13.

Differential diagnosis

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Differential diagnosis

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 Keratocystic odontogenic tumor

 Orthokeratinized odontogenic cyst

 Unicystic ameloblastoma

P.507~509 in Oral and Maxillofacial Pathology, third edition 74

Our case Keratocystic odontogenic tumor

Gender Male Male > female

Age 20 y/o 10~40 yrs (60%) V

Site Mandible (third molar) Posterior Mandibular,

Mostly molar area(49%) V

S/S No Usually asymptomatic

Large: pain, swelling or drainage.

V

size 1 x 2 cm in diameter varies X-ray features Well-defined unilocular

ovoid shaped radiolucency with a sclerotic margins

Well-defined unilocular radioluceny with smooth and often corticated margin

25~40% unerupted tooth involved

V

Clinical features Unknown Usually asymptomatic V

P.683~686 in Oral and Maxillofacial Pathology, third edition

Our case Orthokeratinized odontogenic cyst

Gender Male Male V

Age 20 y/o Young adult V

Site Mandible (third molar) Posterior mandible V

S/S No No V

Size 1 x 2 cm in diameter Less than 1 cm to greater than 7 cm in diameter

V X-ray features Well-defined unilocular

ovoid shaped radiolucency with a

sclerotic margins

Unilocular RL, but occasional examples have been multilocular.

Most often involve an unerupted mandibular

third molar

Clinical features Unknown Usually asymptomatic ₇₅

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Our case Unicystic ameloblastoma

Gender Male None

Age 20 y/o Young age, average 23

Site Mandible (third molar) Post .Mandible

V

S/S no Nil

size 1 x 2 cm in diameter Average size 4.3 cm~6.3 cm X-ray features Well-defined unilocular

ovoid shaped radiolucency with a sclerotic margins

Well-defined, smooth, unilocular

radiolucency with corticated margin V

Clinical features Unknown Color: pink

Pain (-) V

P.702~710 in Oral and Maxillofacial Pathology, third edition

Keratocystic odontogenetic tumor (suspected Gorlin syndrome)

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KCOT and Gorlin Syndrome

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 Etiology and Pathogenesis

─ A distinctive form of developmental odontogenic cyst that deserves special consideration because of its specific histopathologic features and clinical behavior

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 Clinical features

 Sex predilection:♂≥♀

 Age predilection : 10~40 years old (60%)

 Racial predilection: None

 Site predilection: Mandible(60~80%)

 Symptom and Sign

1. 多半沿著anterior-posterior方向長，就算是很大的 lesion也不會有expansion

2. 用針筒抽取病灶，可抽出草綠色或黃綠色的液體

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Anterior Premolar Molar

Maxilla 13% 2% 20%

Mandible 9% 7% 49%

 Radiographic Features

 通常是一個well difined且有sclerotic bone圍繞的unilocular RL，25%~40%包裹著一個impacted tooth

 比起dentigerous cyst跟radicular cyst，KCOT比較不會造成鄰 接牙齒的牙根吸收

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 Histopathologic Features

 可見cyst的三種特徵：cyst wall、lining epithelium、

lumen/cavity

 Cavity中可見到很多類似keratin的東西

 Lining epithelium：

1. 均勻厚度為6~8層的stratified squamous cell 2. epithelium 與wall接面平坦，rete ridge不明顯

3. basal cell呈現柵狀排列(palisaded)，為cuboidal或columnar 4. 最靠近cavity的cell為parakeratinizing，呈現wavy appearance

 若發炎狀況發生，上皮會出現rete ridge且parakeratinizing和palisaded basal cell都消失

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 Etiology and Pathogenesis

 為體顯性遺傳(autosomal dominant)

 與第九對染色體的PTCH(一種tumor supressor gene)基因的 變異有關

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 Clinical Features

 頭圍增加(increased cranial circumference)

 眼距變大(hypertelorism)

 輕微下顎前突(mandible prognathism)

 表皮多發的basal cell carcinoma，特別是在中臉部，30歲前 發生

 手掌或腳掌有凹陷(Palmar and plantar)

 女性有卵巢纖維瘤(Ovarian fibroma)

 肋骨分叉或外展(rib bifid or splayed)

 多發性的odontogenic keratocyst：與一般OKC比，p53和 cyclinD1(Bc1-1)過度表現

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 治療照護

戈林症候群需要有經驗的專科醫師來進行治療及追蹤照護，

重點如下：

* 早發的角化腫瘤藉手術切除。

* 基底細胞瘤早期治療：確實根除，並保留正常組織避免對 外形的損壞

* 卵巢纖維瘤之手術治療：需保留卵巢組織。

* 預防主要症狀：

---避免過度暴露於陽光底下。

---不使用放射治療 (如X射線)，以避免提高治療部位發生多發 性基底細胞瘤的風險。

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* 追蹤監控：

---兒童階段需定期測量頭圍。

---由於患者罹患髓質母細胞瘤的風險高，滿週歲以內即需開 始每半年做一次發展評估及身體理學檢查。

---滿8歲後，每12~18個月做一次牙科orthopantogram檢查，目 的在於及早發現口腔角化囊腫。

---至少每年做一次全身皮膚視診 (skin examination)。

* 對有罹病風險的家人進行評估：由於戈林症候群在照護上 需針對併發症 (如兒童髓質母細胞瘤、口腔角化囊腫、基底 細胞瘤) 進行追蹤監控，患者在生活上也需避免陽光的曝曬

，因此建議有罹病風險而尚未出現症狀的家人，應進行基因 檢測，以確認是否罹病。

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Sanctity of life (生命的神聖性)。

1.. Justice(公義原則): 醫師在面對有限的醫療資源時，應以社會 公平、正義的考量來協助合理分配此醫療資源給真正最需要它 的人。

2. Confidentiality (保密原則):醫師對病人的病情負有保密的責任。

3. Veractity(誠信原則):醫師對其病人有「以誠信相對待」的義 4. Nonmaleficence (不傷害原則):醫師要盡其所能避免病人承受

不必要的身心傷害。

5. Autonomy (自主原則):病患對其己身之診療決定的自主權必須 得到醫師的尊重。

6. Beneficence (行善原則)：醫師要盡其所能延長病人之生命且 減輕病人之痛苦。

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• 在《聖經》的第一篇<創世紀>中,上帝告訴以色列人說:「上帝 按他自己的形象造人。」 「你將是神聖的,因為我是神聖的。」

「生命神聖」觀即由此衍生而得。

• 該觀點主張人的生命是無條件的,有價值及神聖的,人繼承了上帝 的品質,包括一切價值的來源-內在的善 (intrinsic goodness),因 此必須受到尊重。

• 藉此瞭解他個人生命的原真,而認知他個人存活在世上的主要工 作和生活的目的,找到個人存在的意義、價值、目的與任務。

 手術的必要性？

→ Dentigerous cyst最佳的治療方式是 sugical excision，將病灶完整的清除

(enucleation)才能將復發率(recurrence rate) 降到最低。

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告知的對象 1. 本人為原則

2. 病人未明示反對時，亦得告知其配偶與親屬 3. 病人為未成年人時，亦須告知其法定代理人 4. 若病人意識不清或無決定能力, 應須告知其法

定代理人、配偶、親屬或關係人

5. 病人得以書面敘明僅向特定之人告知或對特定 對象不予告知

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 對於患者的疾病嚴重程度是否有確實地通知，

盡到告知的義務？

 是否有清楚的向病人說明清楚疾病病程、治

療計畫、預後、風險？

→皆以已告知病人後，經同意才進行手術。

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 是否有先完整瞭解病人的病史?

→治療前有完整蒐集病史資料，並與病患溝 通後擬定進一步的治療計畫

 手術過程中，是否有造成不必要的醫源性的

傷害？

→沒有不必要醫源性傷害。

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 充分說明病情及治療計畫、風險之後，是否 有讓病人充分自主地選擇治療計畫？

→病人及家屬選擇並同意醫師的建議。

 在做全身麻醉以前，是否有說明完整之後再

請病人自主的簽名同意？

→已充分說明並與家屬溝通。

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 做了Excision 後是否有減輕病人的疼痛感？

或是使病人更不舒服？

→有完整去除病灶區域並拍照記錄術後情形。

並告知術後傷口會疼痛，但持續癒合後疼痛 會逐漸緩解

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 在病例撰寫方面(病灶描述,治療計畫,病人態 度)應書寫詳盡， 使治療過程有詳實的記錄及 治療順利。

 在進行治療之前,須請病人簽屬同意書

 應在不違反醫學倫理的原則之下進行治療的

行為

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