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Case Report

102/10/29 Intern C組

林吟品, 廖之綺, 黃筠婷, 廖秀淳 指導醫師: 陳玉昆醫師

暨 口腔病理科全體醫師

(2)

General data

• Name 蔡O O

• Sex : Female

• Age : 26 y/o

• Native : 高雄市

• Marital status : 已婚

• Attending V.S. : O O O 醫師

• First visit :08/13/2013

(3)

Chief Complaint

• Swelling mass over R't maxilla palatal side

2013/08/13

(4)

Present Illness

• This 26 y/o female suffered from swelling mass over tooth 14- 17 palatal side on 08/13/2013 at 全家 dental clinic routine

follow up, and Dr. O O O suggested her to come to our OPD for further evaluation and examination.

• 08/13/2013 This day afternoon, she came to our OPD to

examination, and Dr. O O O arranged CT scan on 08/29/2013.

• On 09/10/2013, she came back our OPD to check CT report, and report noted the lesion was retention cyst

• Dr. O O O arranged OP schedule on 09/27/2013 under GA to remove lesion

(5)

• 2003/11/24 incisional biopsy under LA in O O Hospital

-0.7x0.4X0.2 retention cyst

• 2004/02/05 Excisional biopsy under LA in O O Hospital

-0.8x0.6x0.3 retention cyst

(6)

Past Medical History

• Underlying disease: (+)

1.Liver disease: hepatitis B

• Hospitalization experience: (+) 1. Surgery for maxillar r't cyst 2. Maternity

• Surgery under GA: (-)

• Denied any drug or food allergy

(7)

Intraoral Examination

• Dimension: 2 x 4 cm

• Swelling: Tooth 14-17, palatal side

• Surface: Smooth, non-ulcerated

• Consistency: Rubbery

• Fluctant (+)

• Pain (-)

• Tenderness (-)

• Induration (-)

• Right maxilla , LAP(-)

2013/08/13

(8)

Image finding – Panorex(102/08/13)

No particular finding

(9)

Dental Finding (102/08/13)

Missing:Tooth 18, 28, 48

OD filling:Tooth 14,15,16,17,21,25, 26, 27, 35, 36, 37, 45, 46, 47

post and crown: Tooth 24

(10)

CT scan (102/08/29)

<Axial view> <Coronal view>

There is a soft tissue density lesion invade into sinus floor and nasal area,result in bony destruction, measured up approximately 2cm in diameter.

(11)

Past Dental History

• Experiences of general routine dental treatment

• Attitude:cooperative

(12)

Personal History

• Risk factors related to malignancy - Alcohol drinking (-)

- Betel quid chewing (-) - Cigarette smoking (-)

• Special oral habits: Denied

(13)

Working Diagnosis

(14)

Peripheral Intrabony This case

Mucosal lesion + - +

Induration + - -

Bony expansion - +/-

-

Destruction of sinus

floor

+/- +/- +

Peripheral or Intrabony

Peripheral

(15)

Inflammation

Inflammation This case

Redness + +

Swelling + +

Local heat + -

Pain + -

Cyst or Neoplasm

(16)

Cyst

Cyst This case

Aspiration + undo

Fluctuation +/- +

Well-defined border

+

+

Bone expansion +/- -

(17)

Neoplasm

Benign Malignant This case

Border Well-defined Ill- defined Well-defined

Sclerotic margin + - -

Destruction of bone +/- + ++

Pain - + -

Induration - + -

Lymphadenopathy - +/- -

Progress Slow Fast Slow

Metastasis - +/- No clear evidence

(18)

CT scan (102/08/29)

<Axial view> <Coronal view>

There is a soft tissue density lesion invade into sinus floor and nasal area,result in bony destruction, measured up approximately 2cm in diameter.

(19)

Differential Diagnoses

• Mucoepidermoid carcinoma, low grade

• Polymorphous low-grade adenocarcinoma

• Pleomorphic adenoma

• Acinic cell adenocarcinoma

• Adenoid cystic carcinoma

(20)

Mucoepidermoid carcinoma

Our case Mucoepidermoid carcinoma

Gender Female Slight female or equal

Age 26 20~70

Site Palatal side Parotid gland, minor gland

Lower lip, floor of mouth, tongue, palate and retromolar pad areas

Pain - Early stage: -

Swelling

+ +

Drainage - -

induration - +

Consistency rubbery Firm or hard

Shape Smooth, dome-shaped Dome-shpaed duration 8 years (since 2003

November founded) slow

(21)

Polymorphous low-grade adenocarcinoma

Our case Polymorphous low-grade

adenocarcinoma

Gender Female 2/3 Female

Age 26 60~80

Site Palatal side Exclusively minor salivary gland (65% palate)

Pain - -, occasionally bleeding or

discomfort Swelling

+ +

Drainage - -

induration - +

duration 8 years (since 2003 November

founded) slow

(22)

Pleomorphic adenoma

Our case Pleomorphic adenoma Gender Female Slight female

Age 26 All ages (common in the third to sixth decades)

Site Palatal side Major salivary glands (most in parotid salivary gland, less in sublingual

gland), minor salivary(33%~43%)

Pain - -

Swelling + +

Drainage - -

Consistency rubbery Firm

Induration - +

Shape Smooth, dome-shaped Single irregularly shaped, bosselated surface Duration 8 years (since 2003

November founded) Slow

(23)

Acinic cell adenocarcinoma

Our case Acinic cell adenocarcinoma

Gender Female 60% woman

Age 26 40 (20~70)

Site Palatal side Parotid gland(85%), minor gland(9%)

Pain - -, sometimes + or tenderness

Swelling + +

Drainage - -

induration - +

Shape Smooth, dome-shaped Smooth surface Duration 8 years (since 2003 November

founded) Slow

(24)

Adenoid cystic carcinoma

Our case Adenoid cystic carcinoma

Gender Female Equal or slight female

Age 26 Middle age adult

Site Palatal side Major and minor salivary gland ( ex. palate)

Pain - +, sometimes facial nerve

paralysis Swelling

+ +

Drainage - -

induration - +

Shape Smooth, dome-shaped Smooth surface or ulcerated Duration 8 years (since 2003 November

founded) slow

(25)

Treatment Course

(26)

Treatment Course

• Excisional biopsy, under GA 102/09/27

• Surgical plan:

cyst enucleation+tooth16 extraction

• Follow up: Wound healing and bone density evaluation

(27)

• 102/08/13- 1. Taking pano

2. Arrange CT scan on 102/08/29

3. Arrange CT scan report on 102/09/10

• 102/08/29-

-CT scan by OOO醫師

• 102/09/10 1. CT report

2. EKG diagnosis: normal

3. Arrange OP under GA on 102/09/27 : Cyst enucleation+tooth16 extraction

(28)

• 102/09/25 Admission

• 102/09/27 OP Day

• 102/09/30 Discharge

• 102/10/07

Suture remove

(29)

• 102/09/27 OP day bone tumor excision

(and simple odontectomy of tooth 38)

(30)

• 102/10/14

F/U+開立診斷證明書

• 102/10/15

Pathology report:

Mucoepidermoid carcinoma, low grade

(31)

Pathologic diagnosis

• 102/10/02

The immunohistochemical stains demonstrate:

S-100 protein: negative.

• 102/10/03

Microscopic examination:

CK(+), p63(+), HMB45(-), SMA(-).

• 102/10/07

Microscopic examination:

The histochemical studies demonstrate:

Mucicarmine(+), PAS(+), PASD: focally positive.

(32)

• 102/10/9'13

Pathologic diagnosis:

Oral cavity, gingiva, tooth 14-17 area , excision, mucoepidermoid carcinoma, low grade

( pT1cN0cM0,stage I)

(33)

Discussion

Mucoepidermoid carcinoma

(34)

What’s mucoepidermoid carcinoma ?

• A tumor usually occur in salivary glands

• The most common malignant salivary gland neoplasm

• It can mimic most other tumors of the glands often considered in the differential

diagnosis

(35)

Epidemiology

• Seen throughout all adult age groups

• Most common in 20-70 years of age

• Most common malignant salivary gland tumor in children

• Overall, MEC account for :

2.8-15.5% of all salivary gland tumors

– 10%(US), 1~2%(UK) of major salivary gland tumors – 15~23%(US), 9%(UK) of minor salivary gland tumors

• In the parotid gland they are the most common malignant primary neoplasm

• A slight female predilection has been described

• Radiation therapy to head and neck has been implicated as

a risk factor

(36)

Clinical presentation

• Most frequently arise in the parotid gland

• Painless swelling

• With or without facial nerve involvement

• These tumours can however be found anywhere there are salivary glands

• Overall distribution across various glands is as follows :

• Major salivary glands : ~ 50%

– parotid gland : ~ 40%

– submandibular gland : ~ 7%

– sublingual gland : ~ 3%

(37)

Clinical presentation

• Minor salivary glands : ~ 50%

– Palate : most common

– Retromolar area

– Floor of the mouth – Buccal mucosa

– Lip

– Tongue

– Other: anywhere in the proximal aerodigestive tract, the lacrimal glands and even in the bronchi

• Presentation will depend on the anatomic location

(38)

Pathology

• The tumours are composed of a mixture of : 1. Mucus secreting cells (muco- )

2. Squamous cells (-epidermoid)

3. Lymphoid infiltrate often also present

• Histology will often show clear mucin containing

cells which stain reddish pink with the musicarmine

stain

(39)

Pathology

• Mucoepidermoid tumours are graded histologically into :

1. Low grade :

a. Well differentiated cells with little cellular atypia b. High proportion of mucous cells

c. Prominent cyst formation

2. Intermediate grade : intermediate features 3. High grade :

a. Poorly differentiated with cellular pleomorphism b. High proportion of squamous cells

c. Consists of solid island

(40)

Grading systems

AUCLAIR ET AL (1992) Point value

Intra cystic component<20% 2

Neural invasion present 2

Necrosis present 3

Four or more mitoses per 10 high-power fields 3

Anaplasia present 4

Grade Total point score

Low 0-4

Intermediate 5-6

High 7-14

(41)

Grading systems

Brandwein ET AL(2001) Point value

Intra cystic component<25% 2 Tumor front invades in small nests andislands 2

Pronouced nuclear atypia 2

Lymphatic or vascular invasion 3

Bony invasion 3

Greater than four mitoses per 10 high-power fields 3

Perineural spread 3

Necrosis 3

Grade Total point score

Low 0

Intermediate 2-3

High 4 or more

(42)

Radiographic features

• Radiographic appearances largely depend on grade, making preoperative imaging important in planning and counselling.

• CT

 Low grade tumors

a. Well circumscribed masses

b. Usually with cystic components.

c. Solid components enhance

& calcification is sometimes seen

have appearances similar benign mixed tumor

 High grade tumors

a. Have poorly defined margins b. Infiltrate locally

c. Appear solid

(43)

Radiographic features

• MRI

 Again, imaging is dependent on grade

 Low grade tumors have similar appearances to benign mixed tumor :

a. T1 - low to intermediate signal ; low signal cystic spaces

b. T2 - intermediate to high signal ; cystic areas will be high signal

c. T1 C+ (Gd) - heterogeneous enhancement of solid

components

(44)

Radiographic features

• MRI

(45)

Radiographic features

 High grade tumours on the other hand have lower signal on T2 and poorly defined margins and

infrequent cystic areas.

a. T1 - low to intermediate signal b. T2 - intermediate to low signal

 It is essential to image the cranial nerves with fat saturated post contrast T1 sequences to assess for perineural spread, and as such the base of skull

should be imaged up to and including the cavernous

sinus and inner ear

(46)

Immunohistochemical Analysis

• Enhance accuracy

• Investigate subjects

– Cell nature and differentiation status – Cell proliferation

– Tumor protein expression

Immunohistochemical Analysis of Salivary Gland Tumors: Application for Surgical Pathology Practice

Toshitaka Nagao, Eiichi Sato, Rie Inoue, Hisashi Oshiro, Reisuke H. Takahashi, Takeshi Nagai, Maki Yoshida, Fumie Suzuki, Hiyo Obikane, Mitsumasa Yamashina and Jun Matsubayashi

Acta Histochem. Cytochem. 45 (5): 269–282, 2012

(47)
(48)

Markers of mucoepidermoid carcinoma

• Squamous epithelial cell – p63

– CK14

Cheuk, W. and Chan, J. K. (2007) Advances in salivary gland pathology.

• Prognostic factor – Ki-67

Skalova, A. and Leivo, I. (1996) Cell proliferation in salivary gland tumors.

– p27

Okabe, M., Inagaki, H. et al. (2001) Prognostic significance of p27 and Ki-67 expression in mucoepidermoid carcinoma of the intraoral minor salivary gland.

– MUC1

Handra-Luca, A., Lamas, G., Bertrand, J. C. and Fouret, P. (2005) MUC1, MUC2, MUC4, and MUC5AC expression in salivary gland mucoepidermoid carcinoma: diagnostic and prognostic implications

(49)

Treatment

• Dependent on grade and location

 low grade (well circumscribed) a. wide local excision

b. preservation of the facial nerve

c. without the need for neck dissection or adjuvant radiotherapy

 high grade (poorly circumscribed) a. complete parotidectomy

b. often with sacrifice of the facial nerve

c. neck dissection (as nodal metastases are common)

d. adjuvant radiotherapy

(50)

Prognosis

• Also very dependent on grade

• Low grade tumours having a 90 - 98% survival and a low local recurrence rate

• High grade tumours having 30 - 54% survival and a very high local recurrence rate

• Has a predilection for perineural spread

careful and long term follow-up is required

(51)

Retention Cyst?

Mucoepidermoid Carcinoma?

• What lead to a retention cyst?

 Partial blockage of a duct of the seromucous gland

• Mucoepidermoid Carcinoma may play a role in the obstruction of a salivary gland duct and caused a retention cyst

Kermani W, Belcadhi M, Mani R, Abdelkéfi M, Sriha B, Bouzouita K. (2008)

Parotid retention cyst revealing a mucoepidermoid carcinoma

(52)

醫學倫理討論

(53)

醫學倫理

• 醫學倫理:一種道德思考、判斷和決策,

以倫理學的觀點出發,以期能做出對病人 最有利益、最能符合道德倫理規範的醫療 決策

(54)

Tom Beauchamp &James Childress 六大原則- 1979

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

2. 誠信原則(Veractity):即醫師對其病人有「以誠信相對待」

的義務。

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

4. 不傷害原則(Nonmaleficence):即醫師要盡其所能避免病人 承受不必要的身心傷害。

5. 保密原則(Confidentiality),即醫師對病人的病情負有保密 的責任。

6. 公義原則(Justice),亦即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。

(55)

行善原則(Beneficence)

• 行善原則包括不傷害原則的反面義務(不應該做的 事)和確有助益的正面義務(應該做的事),包括維 護和促進病人的健康、利益和福祉,為基本倫理 原則,也是醫護人員的基本義務

• 臨床意義

(1) 勿施傷害:不得故意對他人施予傷害或惡行 (2) 預防傷害:應該預防傷害或惡行

(3) 移除傷害:應該移除傷害或惡行

(4) 維持善行:應該致力於行事或維持善行

(56)

誠信原則(Veractity)

• 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?

• 對於病人疾病嚴重程度是否有誠實的通知,

盡到告知的義務?

(57)

自主原則(Autonomy)

• 一位具理性思考能力的病人,在完全瞭解 醫療處置方針的利弊得失下,有權決定自 己的行為,包括決定及選擇醫療專業人員 和治療方式

• 臨床意義

(1) 病人之自主行為不應遭受他人之操控或 干預

(2)指醫療人員應提供充分且適當之資訊,以 促成病人針對診療方式主動作一抉擇

(58)

不傷害原則(Non-maleficence)

• 原則:

不殺害病人、不能侵害病人權益和福祉以及 平衡利害得失,使痛苦減到最低

• 臨床意義

(1)醫療上是必須的,或是屬於醫療適應症範 圍,因所施行的各種檢查或治療而帶來的 傷害應符合不傷害原則

(2)權衡利害原則→ 兩害相權取其輕 (3)保護病人的生命安全

(59)

保密原則(Confidentiality)

• 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

(60)

公義原則(Justice)

• 原則:

強調資源合理分配、賞罰分明以及合乎正義 之事。醫療上公平原則指基於正義與公道,

以公平合理的態度來對待病人、病人家屬 和受影響的社會大眾

• 臨床意義

(1) 公平地分配不足的資源 (2) 尊重病人的基本權利

(3) 尊重道德允許的法律及法律之前人人平等 (4) 先來先服務與急重症優先

(61)

臨床案例討論

原則 臨床討論

行善原則(Beneficence) 是否施行的治療是出自善意,是對病患的生活品質有

所幫助?

誠信原則(Veractity) 是否有讓病患簽署同意書並詳實說明術後可能併發症

自主原則(Autonomy) 有讓病患簽署同意書並得到病人的支持

不傷害原則

(Non-maleficence)

有沒有讓病患承受非正常手術所造成的傷害 還有我們所做的檢查是否為必要的

保密原則(Confidentiality) 對於病患病情的保密,已及他的資料是否會被使用來

研究用途是否有告知

公義原則(Justice) 是否讓病患有可以接受到完整的治療的權益,以及術

前術後照護有無不足之處

(62)

誠信原則與自主原則

(63)

不傷害原則

(64)

Tx. of OO醫院

• 2003/11/24 incisional biopsy under LA in OO Hospital

-0.7x0.4X0.2 retention cyst

• 2004/02/05 Excisional biopsy under LA in OO Hospital

-0.8x0.6x0.3 retention cyst

• 2004/02/12 dexatin application

• 2004/03/27 F/U

-No evidence of recurrence

(65)

The end

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