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CASE PRESENTATION

SUBMANDIBULA TUMOR

Preceptor:
Dr. Herry Setya Yudha Utama, Sp.B, M.HKes, FInaCS

Arranged By:
Rezky Dwiputra Fellanys
1102013248

CLINICAL CLERKSHIP OF SURGERY DEPARTEMENT

FACULTY OF MEDICINE YARSI UNIVERSITY

ARJAWINANGUN DISTRIC GENERAL HOSPITAL

APRIL 9th – JUNE 30th 2018


CASE PRESENTATION

I. IDENTITY
Date of hospital entry : April 26th , 2018
Name : Mr. A
Age : 18 y.o.
Gender : Male
Address : Arjawinangun
Religion : Moslem
Marital status : Single

II. ANAMNESIS
Main Complaint
Patient complained about lump on the right neck

History of Disease
Mr. A (male) 49 years old, came to Arjawinangun hospital with complaints about there
was a lump on the right neck that arose and began to swell since 6 months ago. Lumps
also feel pain and quite interfere with activities such as eating and drinking. Patients
become difficult to talk and salivate more than usual. Patients deny the presence of pain
in the area of the lump, the lump is never red or swollen heat.

History of Past Disease


The patient said he had never experience the same symptoms before. The patient had
no history of surgery.

History of Family Disease


Mr. S said, there is no family member with the same condition with patient.

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III. PHYSICAL EXAMINATION
a. Present Status
General condition : Mild pain
Awareness : Compos mentis
Blood pressure : 120/80 mmHg
Pulse : 90 x/minute, reguler
Respiratory : 20 x/minute
Temperature : 36,4 o C

Head
Form : Normocephale
Hair : Black, no hair fall
Eye : Anemic conjungtiva (-/-), icteric sclera (-/-), light reflexes (+/+),
isochore pupil right = left
Ear : Normal form, cerumen (-), tympanic membrane intact
Nose : Normal form, septum deviation (-), epistaxis (-/-)
Mouth : Normal

Neck
There is a mass on the right side of the neck measuring 5 cm x 3 cm, colored the
same as surrounding, slippery surface, no ulceration, supple consistency and
firmness, tenderness (-), no KGB enlargement.

Thorax
Lungs – Pulmonary
Inspection : chest is symmetrical
Palpation : fremitus vocal and tactile are symmetrical, crepitation (-),
tenderness (-), rebound tenderness (-)
Percussion : Sonor sound in both lung fields
Auscultation : Vesicular abd bronchial sound in the entire lung field, ronchi
(-/-), wheezing (-/-)

Cor
Inspection : Ictus Cordis not visible

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Palpation : Cordis Ictus palpable
Percussion : cardiac boundaries within normal limits
Auscultation: Cardiac Sound I-II Reg. G (-) M (-)

Abdomen
Inspection : Flat, symmetrical, mass (-)
Palpation : Tenderness (+), rebound tenderness (-)
Percussion : Tympanic sound in four quadrants
Auscultation: Bowel sound (-)

Extremities
Upper Lower
Muscle Tone: normal Muscle tone: normal
Movement : active / active Movement : active / active
Mass :-/- Mass :-/-
Strenght :5/5 Strenght :5/5
Oedema :-/- Oedema :-/-

Genitalia
No abnormalities

b. Localized Status
Regio : Neck
Palpable lump in upper right neck as big as fist fist of adult, hard, difficult to move

IV. LABORATORY EXAMINATION

Hematology :

Hemoglobin 14.6 gr/dL

Hematokrit 35,8%

Lekosit 4.710

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Trombosit 275.000

Eritrosit 5.03 mm3

Index Erythrocytes :

MCV 71.4 fl

MCH 29.0 pg

MCHC 40.6 g/dL

RDW 11.6 %

MPV 10.1 fl

PDW 40.0 %

Diff :

Eosinofil 12.0 %

Basofil 0.5 %

Segmen 66.2 %

Limfosit 15.0 %

Monosit 8.8 %

Luc 2.5 %

Coagulation

Clothing Time (CT) 4 Minute

Bleeding Time (BT) 2 Minutes

Clinic Chemical

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Blood Sugar 107 mg/dL

Immunologi

HBsAg 0.01 S/CO

Anti-HIV Non-Reactive

Thorax Rontgen :
COR does not expand, sinuses and diaphragm normal
Pulmo:
- Hili normal
- Lung strokes increase
- No soft fears
Impression:
- No sign of active pulmo TB
- No heart enlargement

V. DIAGNOSIS
Submandibula Tumor

VI. DIFFERENTIAL DIAGNOSIS


Limfadenopati thyroid gland

VII. TREATMENT
Operation (Resection & Chemotherapy )

VIII. PROGNOSIS
Ad vitam : Bonam
Ad sanationam: Bonam
Ad fungsionam: Bonam

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LITERATURE REVIEW

I. Salivary Glands Anatomy

Salivary glands serve to produce saliva, a fluid found in the mouth and throat.
Saliva contains enzymes that serve to help digestion of food. Saliva also serves to block
the occurrence of infection of the mouth and throat. There are two kinds of salivary
glands, namely the major salivary glands and minor salivary glands.

Mayor Salivary Glands

Picture 2.1 Mayor Salivary Glands (Peter and Margaret, 2003)

Parotid gland, is the largest salivary glands with a size of 5.8 x 3.4 cm. Eighty percent
of these glands are located above m. masseter and mandible, another 20% in retromandibula.
The parrot tail is located at the top ¼ m. sternocleidomastoideus and extends to the mastoid
process. The parotid gland is connected to the oral cavity through the Stensoni ducts and
empties into the buccal mucosa as high as the upper two molars. The cranial nerve VII (facial
nerve) that functions motorically for the facial muscles, enters the parotid gland and divides it
into 2 surgical zones (superficial and deep lobes).
This facial nerve in the parotid gland branches into 5, namely: the temporal branch to
the frontal muscle; branch of zygoma to muscle orbicularis oculi; buccal branches to the facial
muscles and upper lip; mandibular branches to the muscles of the lower lip and chin; the
cervical branch to the platisma muscle. The auriculotemporal nerve, which is a branch of the
mandibularis mandibular nerve, runs parallel to the superficial temporal artery and vein. This
nerve carries parasympathetic fibers to the parotis if the injury will result in Frey's syndrome.

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This nerve also causes the spread of malignant parotid tumors to the cranial and intracranial
bases through its perineuralsheat (Suyatno and Emir TP, 2010).
The arteries adjacent to this gland are the external carotid artery, the internal maxillary,
and the superficial temporalis. The drainage is through the deeply located retromandibular vein
of the facial nerve. While the lymphatic drainage through the lymph nodes located within the
parotid gland and paraparotis (Tjakra, 2010).

Picture 2.2 Anatomy of Head (transversal) (Peter and Margaret, 2003)

The submandibular glands, weigh half of the parotid gland. Located within the
submandibular triangle formed by m. anterior and posterior digly of the belly and the inferior
edges of the mandibular ramus. The facial nerve of the mandibular marginal branch runs
superficially from these and inner glands of m. platisma. The submandibular duct (Wharton's
duct) exits the medial surface of this gland and runs between the m. milohioid (lateral) and
hioglosus and to m. genioglosus. This duct enters the lateral oral cavity of the lingual frenulum.
The lingual nerve feels around Wharton's, while the parallel hypoglossal nerve with the ducts,
runs inferiorly from the ducts (Suyatno and Emir TP, 2010).
The artery that enters the submandibular gland is the submental branch of the facial
artery (the branch of the external carotid artery). The drainage is through a facial vein, which
passes through the lateral surface of this gland. Lymphatic drainage runs to the deep cervical
lymph nodes and jugular chain (Suyatno and Emir TP, 2010).

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Picture 2.3 Salivary Glands of Submandibula and Sublingual (Peter and Margaret, 2003)

The sublingual gland, the most minor salivary gland. Located below the basic mucosa
of the mouth between the mandible and m. genioglosus. The inferior part is m. mylohioid. The
Wharton's duct and the lingual nerve pass through the sublingualis and m. genioglosus. This
gland lacks a capsule, unlike the parotid gland and the submandibular gland. This gland also
lacks the dominant duct, its drainage through approximately 10 small ducts (Rivinus duct) and
empties into the sublingual creases at the floor of the mouth (John and Harri, 2007).

Picture 2.4 Salivary Glands of Submandibula and Sublingual (back view) (Peter and
Margaret, 2003)

The artery supplying this gland is the sublingual branch of the lingual artery and the
submental branch of the facial artery. Lymphatic drainage leading to the submandibular lymph
nodes (Suyatno and Emir TP, 2010).

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Minor Salivary Glands

The minor salivary gland, is a gland different from the major salivary glands, ie has no
duct. The minor salivary glands are concentrated in the buccal, labial, palatal, and lingual
regions. Minor salivary glands may also be found in the upper pool of tonsils (Weber's gland),
tongue base (von Ebner's gland), paranasal sinuses, larynx, trachea, and bronchi. Most of these
glands are present in the palate, upper lip, and cheeks. Most minor salivary glands receive
innervation from the lingual nerve, except for glands in the palate that receive the innervation
of the palatine nerve (Kuppersmith, 1995)

II. Epidemiology
The salivary gland neoplasm is a benign or malignant neoplasm derived from
the salivary gland epithelium, either the major or minor salivary gland. Salivaryal gland
cancer is 5-7% of all malignancy heads, and in the United States there are 2000 to 2500
new sufferers per year, whereas in Indonesia the incidence rate is unknown. Eighty-five
percent of salivaryus gland tumors are present in the parotid gland (the largest salivary
gland), and 75% are benign tumors which are mostly pleiomorphic adenomas (benign
mixed tumors) and with a smaller incidence of a monomorphic adenoma (Wartin's
tumor). While the other major salivary glands such as the submandibularis salivarius
gland are 50% incidents as malignant / cancerous tumors, and in sublingual sublosts
almost all are malignant / cancerous (Tjakra, 2010).
Said to be the etiology of salivary gland cancer is exposure to radiation
especially the type of mucoepidermoid carcinoma. While adenocarcinoma occurring in
the nasal cavity or sinus paranasales (especially sinus ethmoidalis) is associated with
exposure to "wood dust", and is often found in wood industry workers (Tjakra, 2010).
The incidence of salivary gland cancers continues to increase with age, and the
incidence of this cancer in patients <16 years is 2% (Futran, et al, 2009).
The likelihood of exposure to male salivary glands equals women. It is rare in
children but the frequency of malignancy is more common in children. Approximately
35% of salivary gland tumors in children are malignant, the most common type is
mucoepidermoid carcinoma. Tumor / cancer of the salivary gland is often correlated
with gender, which results from no predilection of sexual data except in monomorphic
adeoma (Warthin tumor) found 5 times more in men (Tjakra, 2010).

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III. Classification of Tumor
Clinical Stadium Classification (Stevenson, 2011)
Clinical stage determination was established on the basis of TNM from AJCC
in 2002, with revisions that have been made several times. This is due to new
technology in the diagnosis and staging of cancer, the presence of new painting
techniques with monoclonal antibody and molecular biology technique (PCR / RT-
PCR)
The proposed TNM classification is in tumor / malignancy of the parotid
salivarius glands, which can also be used in other salivary gland malignancies.

Tumor (T)
Primary tumors can not be evaluated
T0-no signs of tumor
T1- tumor with size ≤2cm without extraparenkim extension
T2- tumor> 2 cm and ≤4 cm without extraparenchyme extension
T3- tumor> 4cm with extension to extraparenkim
T4- infiltration of the tumor so that the skin, mandible, ear canal, and / or facial nerve
T4b- infiltration of the tumor to the base of cranium and / or pteryoid muscle

Nodul (N)
Nx-metastatic regional lymph nodes can not be evaluated
N0- there is no metastasis from regional lymph nodes
N1- ipsilateral single metastasis from regional lymph nodes, with ≤3 cm in size
Single ipsilateral α-metastasis from regional lymph nodes, with ≤3 cm in size but ≤6
cm
Ipsilateral multiple-metastasis n2b- metastasis from regional lymph nodes, with ≤6 cm
in size
Bilateral or contralateral n2c- metastasis of regional lymph nodes, measuring ≤6 cm
N3- there is regional lymph node metastasis, with size> 6 cm

Metastasis (M)
Mx- distant metastases can not be evaluated
M0- there is no distant metastasis
M1 - there is a distant metastasis

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Stadium Histopatology WHO (Tjakra, 2010)
Benigna
 Pleiomorphic adenoma(Benign Mixed Tumor)
 Monomorphic adenoma
 Papillary cyst-adenoma lymphomatosum (Warthin Tumor)
Maligna
 Mucoepidermoid carcinoma
 Acinic cell carcinoma
 Adenid cystic carcinoma
 Adenocarcinoma
 Epidermoid carcinoma
 Small cell carcinoma
 Lymphoma
 Malignant mixed tumor
 Carcinoma ex pleiomorphic adenoma

IV. Risk Factor


Age, usually patients diagnosed with this disease, between the ages of 50 and 60 years.
Radiation exposure, especially in patients undergoing treatment with radiation in the
head or neck area, increases the risk of salivary gland tumors. Or those working in
places where there is radiation exposure also increase the risk of salivary gland tumors.
Family history, in patients with previous family history, has a higher risk for salivary
gland tumors (John and Harri, 2007).

V. Diagnosis (Tjakra, 2010)


Anamnesis
 Lumps of the parotid, submandibular and oral mucosal glands (palate, sublingual)
 A lump in the parotid gland is usually located preauricular, causing the ears to rise,
pain or not (associated with N. trigeminal cranialis), the presence or absence of
bell's palsy associated with parotid malignancy.
 Paralysis n. fascialis is present in approximately 2-3% of parotid malignancies.

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 Dysphagia, throat pain, and hearing loss are associated with malignant profundus
lobes with extension to the oropharynx.
 Paralysis n. glossopharyngeus, n. vagus, n. hypoglossus, n. accessories, truncus
sympathicus (horner syndrome) associated with parotid malignancy with extension
on the nervi.
 The presence of KGB neck enlargement, especially at levels I, II, and III are usually
associated with malignant metastases derived from salivary glands.
 Progression of disease. The rate of growth associated with malignant grading and
tumor size (cancer cells doubling time)
 Risk factors: exposure to radiation and exposure to leather plant waste or sawdust.

Physical Examination
 Generalist status (examination from head to foot and vital signs)
 General State (anemia, icterus, cough / shortness of breath, paresis of the
extremities)
 Performance status (Karnofsky score)
 Metastatic signs of KGB, lung, liver, bone or vertebra
 Local Status
i. Inspection the location of the tumor, the shape of the tumor, the
surrounding organs, and the condition of the skin or mucosa above the
tumor. On the neck, lifted ear lobules / lobules, KGB enlargement. Intra oral,
is there a blockage of the stensen's duct (stone, stricture), mucosal bulge in
the parapharingeal area or tonsil / uvula cramps.
ii. ii. Palpation bimanual palpation is performed to assess the consistency,
surface, mobility, size, limit and tenderness. Besides, the function of n. VII,
VIII, IX, X, XI, XII.
 Regional Status, palpation of KGB neck at all levels, especially at all levels
especially upper level (level I, II, III), both ipsilateral and contralateral. If there is
an enlargement determine the location level, the largest size of KGB, the number
and mobility.

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Investigations
Radiological Examination
Radiological examination for diagnosis (on indication)
• Plain jaw image to determine whether or not the jaw bone (mandibular / maxilla) is
involved in the malignant process of this salivary gland
• To perform a differential diagnosis of jaw bone cyst, jaw bone malignancy (Ewing
Sarcoma, osteo sarcoma) and salivary gland tumors (parotid and submandibularis)
• Sialography, made when there is a differential diagnosis of a parotid cyst or
submandibula. This examination is needed to see the image of the stenson's duct and
its branches. With sialography can be seen there is narrowing or blockage of the duct,
the shadow of the narrowed and fibrotic. Can also be seen whether the duct structure is
pushed or not by a tumor mass.
Radiological examination for staging
• Chest X-ray to see pulmonary metastases.
• Abdominal ultrasound
CT Scan / MRI
• Especially for large salivary gland tumors, with limited mobility.
• Important for surgical and operability approaches, eg in parotid tumors of the
profundus lobes and extension into the oropharynx.
Metastasis on KGB neck, extension, sometimes to see extracapsular extension.

Biopsy (Albar, et al, 2003)


 FNAB, not yet a standard check, its accuracy varies (60% - 90%). In the FNAB
submandibula tumor is very helpful if the tumor positive.
 Cut frozen, done on operative specimens for operable tumors. In parotid tumors,
frozen cuts are performed from superficial parotidectomy specimens,
submandibular tumors and minor salivary glands from excision specimens.
 Excisional biopsy, recommended for sublingual tumors and small salivary gland
tumors, is performed in the form of definitive surgery (wide excision). When an
inoperable tumor is performed an incisional biopsy. An incisional biopsy should be
avoided in an operable tumor to prevent tumor spillage, tumor damage and facial
nerve injury.

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VI. Managment
Submandibular Gland Tumors
For benign tumors, excision of the submandibular gland is for diagnosis and
curative of course with frozen cut confirmation. If the frozen benign operation is
completed, if malignant is continued the submandibular dissection (Excision of
Lymphatic Level I structure) and frozen. If the lymph nodes contain metastasis
followed by radical neck dissection. The sequence of actions is performed when there
is no clinical neck lymph node enlargement (N0). If no bone is involved with N0
extended supraomohyoid dissection includes removal of the glandular bed, muscles
and nerves around it. If clinical lymph nodes are palpable, neck dissection (Albar, et
al, 2003) is modified.
Neck dissection is performed when there is enlarged clinically naïve (n-
positive) lymph nodes. If there is mandibular infiltration, composite resection
(mandibulectomy and one-sided neck dissection) is performed. As with parotid
tumors, removal of the hypoglossal nerve and lingual nerve is only done if the
macroscopic has infiltrated the tumor and local extension to the surrounding tissue
(eg, the base of the mouth, the tongue) requires a more radical excision (Suyatno and
Emir TP, 2010).

Radiation
Radiation as primary therapy is indicated in cases of inoperable salivary gland cancer
and as a post operative adjuvant in high grading cancer or recurrence cases. Adenoid cystic
carcinoma, high grade mucoepidermoid carcinoma, high grade adenocarcinoma, squamous cell
carcinoma and lymph node metastasis are the specific cases requiring adjuvant radiation.
Adjuvant radiation is also indicated in tumors attached to the nerves (facial, lingualis,
hypoglossus and assesorius), residual carcinoma, lobus profundus carcinoma, there is a
macroscopic or microscopic tumor residue and in T3 or T4 stage cancer. In cases of recurrent
or macroscopic pleomorphhic adenomas, tumor spoillage may be given post operative
radiation. As adjuvant radiation can decrease local recurrence and increase survival rate, local
recurrence decreases from 54% to 14%. The dose of radiation on the primary tumor and
covering the incision site is 50-70 Gy (Tjakra, 2010).
Adjuvant adjuvant radiation post-neck dissection (regional / neck) is indicated in all high
grade malignancy, T3 or T4 stage cancer, there is a lymph node containing more than 1
metastasis, there is an extra growth of capsule or lymph node diameter over 3 cm.

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Chemotherapy
Chemotherapy can not be used as a primary therapy for curative purposes in salivary
gland cancer. Data on the role of chemotherapy in cancer is still limited. Chemotherapy may
be given as an adjuvant or palliative in metastatic cases. Response to chemotherapy generally
ranges from 10-30%.
Doxorubicin and 5-fluorouracil were inferred in response to a retrospective study (in
adenoid cystic carcinoma) but were not proven prospectively. Cisplatin, paclitaxel, vinorelbin,
epirubicin and mitoxantrone averaged a response of 10-20% in prospective studies with
metastatic or recurrent cancer samples. The combination of chemotherapy containing cisplatin
or anthracycline (cyclophospamide / doxorubicin / cisplatin / vinorelbin, cisplatin / 5-FU) will
increase the average response to 20-30% with tolerable toxicity (Tjakra, 2010).

VII. Prognosis and Follow Up


Prognosis
In salivary gland cancer, overall 5 years survival is 70-90% at low grading and
20-30% in high grading tumors. Total recurrence and distant metastases vary from 15%
to 20% and commonly occur in the perineural invasive karsioma (adenoid cystic
carcinoma). Survical 5 years on benign tumors reaches 100%, high risk for recurrence
in patients who get adequate surgery.

Submandibular Gland Tumor


Byers et al. Reported the results of selective radiation therapy on malignant
submandibula tumors, the mean local control was 64% and the survival rate was 50%.
Spiro reported the results of surgery of 129 submandibular gland cancers with
observations of at least 10 years of obtaining a 40% locoregional control and the cause-
specific cure rate for 5 and 10 years were 31% and 22%. The benign submandibula
benign tumor operated in 106 patients and only 2 cases were recurrent (Albar, et al,
2003).

Follow Up
It is recommended every 3 months in the first 3 years after the therapy is
completed, then every 6 months for 5 years and continued once a year for life. At the
patient's annual follow-up examined completely: physical, chest X-ray, abdominal

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ultrasound (liver), and bone scan to determine cancer-free patients or not. Information
to look for at the examination is the length of life (in years and months), duration of
tumor-free intervals, patient complaints, performance status, disease status (cancer-
free, residual, metastatic, cancer or new disease), therapy complications and therapy .
In the adenoid cystic carcinoma that has the potential to spread along the nerve and
metastasize deep into the lungs, a careful imaging examination (MRI and thoracic
photos) should be performed (Suyatno and Emir TP, 2010).

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BIBLOGRAPHY

Albar ZA, Tjindarbumi D, Ramli M, Lukito P, dkk. 2003. Protokol PERABOI. Bandung.

American Society of Clinical Oncology. 2011. Salivary Gland Cancer.


www.cancer.net/cancer-types/salivary-gland-cancer/risk-factor. Diakses tanggal 01
Oktober 2012 jam 2030

Futran N, Parvathaneni U, dan Martins RG. 2009. Malignant Salivary Gland Tumors. In :
Harrison LB, Sessions RB, Hong WK, eds. Head and Neck Cancer: A Multidisciplinary
Approach, 3rd ed. Philadelphia: Lippincott Williams & Wilkins; 589.

John MM dan Harri PA. 2007. Salivary Gland Neoplasms. Emory University Hospital:
Emedicine.

Kuppersmith RB, et. Al. 1995. Minor salivary gland tumour. Baylor College of Medicine,
Dept. of Otolaryngology; hal 1-13.

Licitra L, Cesare G, Franz JP, Jan HS, Paolo B, dan Roberto M. 2003. Major and Minor
Salivary Glands Tumours. Critical Reviews in Oncology/ Hematology 45: 215-225.

Peter SM dan Margaret SB. 2003. Salivary Glands: Anatomy and Pathology. Mosby Chap. 39:
2005-2133.

Stevenson MM. 2011. Major Salivary Glands Cancer Staging.


emedicine.medscape.com/article2047666-overview. Diakses tanggal 01 Oktober 2012
jam 20.30.

Suyatno dan Emir TP. 2010. Bedah Onkologi: Diagnosis dan Terapi. Jakarta: CV Sagung Seto;
hal. 121-147.

Tjakra WM. 2010. Panduan Penatalaksanaan Kanker Solid PERABOI 2010. Jakarta: CV
Sagung Seto; hal 74-95.

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