LAPORAN KASUS ONKOLOGI

TATALAKSANA PSEUDOMYXOMA
PERITONEI DENGAN KANKER SERVIKS
Oleh :
Abdul Gafur
Pembimbing :
dr. SARAH DINA, M.Ked(OG), Sp.OG(K)
DIVISI ONKOLOGI GINEKOLOGI- DEPARTEMEN OBSTETRI DAN GINEKOLOGI
FAKULTAS KEDOKTERAN UNIVERSITAS SUMATERA UTARA
MEDAN-2017
Outline
Pendahuluan
Definisi
Insidensi dan Faktor Resiko
Patofisiologi
Gejala Klinis
Diagnosis
Tatalaksana
Prognosis
Laporan Kasus
 Pendahuluan
Tumor (1884)
Werth
Asites gelatinosa
primer dan implantasi yang melibatkan
Pendahuluan
permukaan peritoneum dan omentum
Definisi
Insidensi
dan Faktor
Resiko
Patofisiologi
Gejala Klinis
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus

Bevan KE, Mohamed F, Moran BJ. Psedomyxoma peritonei. World J Gastrointest Oncol. 2012: 2(1): 44-50.
 Definisi

Pendahuluan Terminologi : tumor musinosum

Definisi
semu pada peritoneum
Insidensi
dan Faktor Definisi : sindroma klinis yang
Resiko
Patofisiologi
ditandai adanya akumulasi
Gejala Klinis musin - musin ekstraseluler di
Diagnosis rongga peritoneum akibat
Tatalaksana
penyebaran tumor primer
Prognosis
Laporan
musinosum
Kasus

Moghaddam SM, Ehteda A. Morris DL. Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic p
t Journal of Rare Disease. 2014: 9; 1-12.
Moghaddam SM, Ehteda A. Morris DL. Pseudomyxoma Peritonei: Uninvited Goblet Cells, Ectopic MUC2. J Glycobiol. 2013: 1(2); 1-12.
Insidensi
Penyakit neoplastik yang jarang
Pendahuluan
Definisi
Insidensi
dan Faktor
Resiko
Patofisiologi
Gejala Klinis
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus

Jivan S, Bahal V. Pseudomyxoma peritonei. Postgrad Med J. 2012: 78; 170-172.

Vogel, 2010
 Faktor Resiko

Pendahuluan Tidak diketahui jelas

Definisi
Insidensi
Studi retrospektif hanya
dan Faktor menunjukkan umur sebagai
resiko
Patofisiologi
faktor resiko yang predominan,
Gejala Klinis rerata usia 59 tahun
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus

Galani E, Marx GM, Steer CB, Culora F, Harper IG. Pseudomyxoma peritonei: the controversial disease. Int J Gynaecol
Cancer. 2003; 13(4): 413-8.
 Patofisiologi

Pendahuluan
Definisi
Insidensi
dan Faktor
resiko
Patofisiologi
Gejala Klinis
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus

Orphanet J Rare Dis. 2014; 9: 71.

Gejala Klinis
Massa abdomen/
pelvik
Nyeri abdomen
Demam
Anorexia
Nausea/vomitus
Obstruksi parsial
traktus GI
 Tumor Marker

Pendahuluan CEA meningkat pada 75%

Definisi
kasus
Insidensi
dan Faktor CA 19.9 meningkat pada 58%
resiko
Patofisiologi
kasus
Gejala Klinis CA 19.9 deteksi rekurensi
Diagnosis
Tatalaksana
MUC-2 appendiceal origin
Prognosis
Laporan
Kasus

Kamal SM. Pseudomyxoma Peritonei: a review. Faridpur Med. Coll. J. 2012;7(2):88-92

Teknik Pencitraan
CT Scan dengan
kontras IV
USG Abdomen
Foto Polos
Abdomen
Temuan Intraoperatif

Pendahuluan
Definisi
Insidensi
dan Faktor
resiko
Patofisiologi
Gejala Klinis
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus

P.H. Sugarbaker et al. / Gynecologic Oncology Reports 10 (2014) 58

Temuan Intraoperatif

Pendahuluan
Definisi
Insidensi
dan Faktor
resiko
Patofisiologi
Gejala Klinis
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus

P.H. Sugarbaker et al. / Gynecologic Oncology Reports 10 (2014) 58

Gross Macroscopic

Pendahuluan
Definisi
Insidensi
dan Faktor
resiko
Patofisiologi
Gejala Klinis
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus

K.R. Lee et al. Tumours of the Ovary and Peritoneum

 Differensial Diagnosis

Pendahuluan
Definisi
Insidensi
dan Faktor
resiko
Patofisiologi
Gejala Klinis
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus

K.R. Lee et al. Tumours of the Ovary and Peritoneum

Histopatologi

Pendahuluan Keganasan derajat rendah

Definisi
Insidensi
Asal tumor dari
dan Faktor apendiks/ovarium
resiko
Patofisiologi
Kistadenoma musinosum
Gejala Klinis Mukokele
Diagnosis Kistadenokarsinoma musinosum
Tatalaksana
Prognosis
Laporan
Kasus
 Tatalaksana

Pendahuluan
Surgical Debulking Procedures
Definisi Intraperitoneal / systemic
Insidensi
dan Faktor chemotherapy
resiko Platinum based
Patofisiologi
Gejala Klinis
Terapi Kombinasi
Diagnosis
Tatalaksana
Prognosis
Laporan
Kasus
 Tatalaksana

Pendahuluan Terapi kombinasi

Definisi Peritonektomi dengan
Insidensi
dan Faktor elektrosurgikal
resiko Intraoperative hyperthermic
Patofisiologi
intraperitoneal chemotherapy
Gejala Klinis
Menyingkirkan penyakit residual
Diagnosis
Tatalaksana
mikroskopis / minimal
Prognosis
Hipertermia meningkatkan

Laporan efektivitas obat

Kasus Early post operative
intraperitoneal chemotherapy
Sugarbaker. New standard of care for appendicial epithelial neoplasms and pseudomyxoma peritonei syndrome.
Lancet Oncol 7 (1): 69-76
 Prognosis

Pendahuluan Gough and colleagues 32%

Definisi
10-year survival (n= 56) pada
Insidensi
dan Faktor serial debulking dan selective
resiko
intraperitoneal radiotherapy
Patofisiologi
Gejala Klinis
atau kemoterapi.
Diagnosis Complete cytoreduction +
Tatalaksana
HIPEC 80% 5 year survival
Prognosis
Laporan
pada pasien dengan lesi low
Kasus grade
Gough DB, Donohue JH, Schutt AJ. et al. Pseudomyxoma peritonei: Long-term patient survival with an aggressive regional approach. Ann
Surg 1994;219:112- 119
CASE REPORT
Case Report
Mrs.N, a 47 years old P4A0, youngest child 17 years old,
married 1x aged 22 years old, married to Mr.T 50 yo
married 1x at 25 yo, entrepreneur, presented to gyne-
oncology outpatient clinic Haji Adam Malik General Central
Hospital on July 4th 2016
Presenting complaint : Vaginal bleeding
History of present complaint :
Experienced by patient for the past 1 year, with the volume
2 3 changing pads per day, blood clots (+). History of
leucorrhea (+), itchy (-), foul smell (+), history of post coital
bleeding (+). History of abdominal pain (+), pelvic pain (-).
Micturition and within normal limits. Defecation difficulties
(+). Patient was scheduled for CT Simulator on september
2015 but the patient didnt attend the schedule.
Case Report
Menstrual history :
Menarche at the age of 13 years old with regular 28 - 30
days cycle, lasting for 5 7 days. She had no
dysmenorrhoea, dyspareunia or menorrhagia.
Past medical history : Not found
Past surgical history : Not found
Family history : No history of gynecological malignancy.
Present State:
Weight : 47 kg
Alertness : CM Anemic : - Height : 153 cm
BP : 120/70mmHg Icteric :- BMI : 20.07 kg/m2
HR : 88 x/i Cyanosis : -
RR : 20 x/i Dyspnoe : -
T : 36,80C Oedem : -
 Case Report
Local examination :
Head : Eye conjunctiva palpebra inferior anemic (-)/(-)
Ear/Nose/Mouth within normal limit
Neck : lymph node enlargement (-)
Thorax : symmetrical fusiform
Lung Breath sound : vesicular normal , additional sound :
(-)
Heart Heartsound : S1, S2 (normal), murmur (-), gallop (-),
Abdomen : Relaxed, no mass was palpable, The liver and spleen
were not enlarged and the kidneys were not ballotable. The bowel
sound was present and revealed normal
Ext. Sup/inf : within normal limits
Case Report
Gynecological examination
Inspeculo : exophytic mass in cervix with 1/3 distal
vaginal involvement.
RVT : exophytic mass in cervix measured 5x3x5 cm,
with 1/3 distal vaginal involvement. Parametrium
left/right nodular reaching the pelvic wall. Smooth
rectal mucosa.
Histopathological examination :
September 29th2015: Adenocarcinoma endocervix
Working Diagnosis: Stage IIIB Cervical
carcinoma
Planning: Chemoradiation
Case Report
Chest X-ray result July 15th 2016
Right pleural effusion
Consultation result from Pulmonology
Department :
Minimal right pleural effusion ec cervical
cancer
Planning according to Obgyn department,
treat underlying disease.
Case Report
August October 2016
August 1st 2016. CT Simulator was performed
September 5th 2016. External pelvic irradiation was
started.
Patient was on pain management medication to treat
severe cancer pain.
September 19th 2016. Patient was admitted to oncology
ward for blood transfusion, natrium substitution and
albumine substitution. External pelvic irradiation 22x.
September 21st 2016. Patient felt abdominal enlargement
for the past three weeks accompanied by nausea,
constipation and abdominal discomfort. Signs of ascites
was found in physical examination. Patient planned for
Abdominal CT Scan with IV contrast.
Case Report
CT Scan Whole Abdomen with IV Contrast
Results :
Massive ascites
Cirrhosis hepatic appearance not found
Both adnexa showed no mass
Ileus obstructive not found
September 29th 2016. Patient was consulted to
Gastroenterology Division of Internal Medicine
Department with Paralytic ileus, advice :
Abdomen ultrasound and tapping ascites with
cytologic analysis found no malignant cells.
Case Report
October 1st 2016. Patient was assessed
by gyne-oncologist to underwent the
second ascites punction, a mucinous
fluid was found, patient diagnose with
Pseudomyxoma peritoneum, pelvic
irradiation was continued.
Tumor Board result :
October 5th 2016.
CT Scan reviewed : adnexal mass (+)

Working Diagnosis : Pseudomyxoma

peritonei
Patient was scheduled for Laparotomy

Complishment of external pelvic

irradiation.
Case Report
External pelvic irradiation was finished
25 x at October 8th 2016, patient was
never receive cisplatin weekly
chemotherapy due to impaired renal
function.
October 13th 2016. Laparotomy was
performed.
Surgery Report October 14th 2016

Midline incision
Mucinous fluid evacuated 9000 cc
Septate mass was found from right ovarium, mass
was already ruptured, measuring 12 cm
Liver, spleen no abnormalities found
Peritoneum wall thickened
Mucinous mass was evacuated, salphingo-
oophorectomy dextra was performed.
Abdominal cavity washed with normal saline and
aqua.
Abdominal wall was sutured layer by layer.
Case Report
October 18th 2016. Patient was complaining leg pain and
leg swelling. Consultation results from Haematology
Oncology Medic Division of Internal Medicine
Department suggest a diagnosis of Deep
VeinThrombosis confirmed by ultrasound and High Risk
Thrombosis, patient was administered Lovenox injection
0.6 cc/day.
October 20th 2016 Radiotherapist advice a CT Scan
examination due to ascites findings that may contribute
to the consideration of the next procedure whether
performing External Irradiation Booster or
Brachytherapy. Patient was prepared for Abdominal CT
Scan scheduled and the result was massive ascites
findings.
Case Report
Abdominal CT Scan with IV contrast October 24 th 2016
Results :
Massive ascites
Bilateral pleural effusion
No mass in both adnexa
Obstructive ileus not found
Liver cirrhosis not found

Histopathology Result October 26th 2016

Conclusion : Ovarium mass? : Papillary Adenocarcinoma

October 27th 2016. Patient was scheduled for External

Booster Irradiation 10 x 2 Gy
Gyne-Oncologist Assessment :

November 11th 2016.

Diagnosis: Stage IC Ovarian carcinoma + Stage
IIIB Cervical Cancer + High risk thrombosis
Planning :
General condition improvement
Cervical cancer : brachytherapy
Ovarian cancer : chemotherapy if karnoffsky score >
70 with dosage of Carboplatin AUC 5 + Paclitaxel
175 mg/m2 6 cycles
November 11th 2016. Chemotherapy
Carboplatin-Paclitaxel cycle I was performed.
Case Report
November 17th 2016. Patient was discharge from
the hospital with an appointment to oncology
outpatient clinic for wound care.
December 5th 2016. Patient was planned for
secondary heacting d/t wound dehiscense.
December 7th 2016. CT Simulator for external
booster was performed.
December 16th 2016. Second cycles of
chemotherapy Carboplatin Paclitaxel.
January 5th 2016. Patient was admitted to the
gyne-oncology ward from the outpatient clinic for
blood transfusion and third cycle of chemotherapy.
CASE ANALYSIS
Case Problem Analysis
Mrs N, 47 years of age, P4A0, Patient with risk factor for cervical carcinoma: multiparity,
housewife,married 1x at 22 years husband suspicious for promiscuity
of age wife of Mr.T, married 1x,
entrepeneur
Working Diagnosis: This was advance stage cervical cancer, the management is
Stage IIIB Cervical carcinoma chemoradiation
Planning: Chemoradiation
CT Scan Whole Abdomen with IV Tumor board results found adnexal mass. CT Scan is the best
Contrast Results modality for assess pseudomyxoma peritonei. Computed
Massive ascites tomography usually shows four basic patterns:
Cirrhosis hepatic appearance (1) posterior displacement of the intestines with numerous low
not found density masses and calcifications,
Both adnexa showed no mass (2) diffuse peritoneal infiltration appearing similar to ascites with
Ileus obstructive not found septated fliud pockets filling the pelvic cavity,
(3) intrahepatic low density attenuated lesion,
(4) scalloping of intra-abdominal organs due to extrinsic pressure
of adjacent peritoneal implants.
September 29th 2016. Patient was The most common presenting symptoms are
consulted to Gastroenterology symptomatic pelvic or abdominal mass and an increase in
Division of Internal Medicine abdominal girth.Some reports also note symptoms of painful
Department with Paralytic ileus sensation in the abdomen, fever, anorexia, nausea, vomiting and
weight loss may be associated with pseudomyxoma peritonei.
Abdomen ultrasound and tapping Diagnosis is seldom absolute until a laparotomy is
ascites with cytologic analysis performed, despite the presence of a distended
found no malignant cells. abdomen with non-shifting ascites on physical
Mucinous fluid found. examination. Analysis of the ascetic fluid is often
disappointing. Most of the time only low quantities of
mucous can be harvested with a few cells. These cells
often have benign features. The lack of malignant cells
in the ascites does not exclude a carcinomatosis
Surgery Report October 14 2016 Pseudomyxoma peritonei (PMP) is characterized by
th

Midline incision copious amounts of mucinous ascites and mucinous

Mucinous fluid evacuated peritoneal implants. Mucinous ovarian tumors
9000 cc associated with PMP are bilateral in 80% of cases with
Septate mass was found from a mean diameter of 7 cm. When unilateral, there is a
right ovarium, mass was right-sided predominance. In disseminated peritoneal
already ruptured, measuring adenomucinosis (DPAM), the ovaries are often cystic
12 cm and usually display mucoid surface, surface nodules,
Liver, spleen no abnormalities or implants. In peritoneal mucinous carcinomatosis
found (PMCA), the ovaries can appear similar but are more
Peritoneum wall thickened often solid. In 75% of patients with PMP, there is gross
Mucinous mass was or microscopic evidence of rupture of the appendiceal
evacuated, salphingo- tumor. Appendiceal rupture can be very small, can
oophorectomy dextra was heal or can be overlooked as a result of inadequate
performed. sampling and these are the best explanations for most
Abdominal CT Scan with IV contrast (Follow The series reported by Wertheim et al. also
Up) October 24th 2016
Results :
Massive ascites
Bilateral pleural effusion
No mass in both adnexa
Obstructive ileus not found
Liver cirrhosis not found
November 11th 2016.
Carboplatin-Paclitaxel Ist
performed.
noted that 40% of the patients with
borderline tumors had died or developed a
recurrence after a median follow-up interval
of 3 years and therefore they agreed with
Kaern et al. that when pseudomyxoma
peritonei is found in association with these
tumors, the prognosis may be worse than
when pseudomyxoma peritonei is not
present.
Chemotherapy Postoperative intraperitoneum
chemotherapy
cycle was
and intravenous chemotherapy are also
reasonably effective, particularly for ovarian
carcinomas. Cisplatin-based regimens have
become the standard of treatment in cases
of ovarian epithelial neoplasms and some
articles have reported using single cisplatin
November 11th 2016. Postoperative intraperitoneum chemotherapy and
intravenous chemotherapy are also reasonably
Chemotherapy Carboplatin-
effective, particularly for ovarian carcinomas.
Paclitaxel Ist cycle was Cisplatin-based regimens have become the standard
performed. of treatment in cases of ovarian epithelial neoplasms
and some articles have reported using single
cisplatin or cisplatinum-based regimens to treat
pseudomyxoma