An 81-year-old woman presented with a pelvic mass. The computed tomography scan showed a non-

calcifiedsolidcysticmass,10.4x11.5x6.7centimeters.Sheunderwent totalabdominalhysterectomy withbilateral



salpingo-oophorectomy,omentectomyandascitic fluidforcytology. Histopathologyreportedpoorlydifferentiated

thyroid carcinoma arising in struma ovarii. Currently she has been free of disease for 5 months from the date of

diagnosis

Atthapon Jaishuen

Mongkol Boonsripitayanon

Pisutt Srichaikul

Wasan Yotchai

Siriraj Medical Journal

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Siriraj Med J, Volume 68, Number 2, March-April 2016 109CaseReportSINTRODUCTION	 	 truma	ovarii	was	first	described	by	Bottlin 	 	 in	1888	and	later	by	Pick	in	19021.	Although 	 	 thyroid	tissue	can	be	detected	in	approxi-mately	15%	of	cases	of	mature	teratoma,	the	latest	World	Health	Organization	classification	remarks	that	the	struma	ovarii	must	contain	at	least	50%	of	 thyroid	 tissue.	Therefore,	 struma	ovarii	 is	diagnosed	 in	only	2.7%	of	ovarian	 teratomas2. Malignant	transformation	occurs	in	approximately	5-10%	of	these	tumors	with	papillary	carcinoma	and	 follicular	carcinoma	being	 the	most	com-mon1.	The	diagnosis	of	thyroid	carcinoma	arising	in	struma	ovarii	should	be	based	on	the	similar	criteria	of	primary	thyroid	gland	disease.	Due	to	Poorly Differentiated Thyroid Carcinoma Arising in Struma Ovarii: Case Report and Review of the LiteraturePisutt Srichaikul, M.D.*, Wasan Yotchai, M.D.**, Atthapon  Jaishuen, M.D.*, Mongkol Boonsripitayanon, M.D.****Department of Obstetrics and Gynaecology, **Department of Pathology, ***Department of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand.ABSTRACT	 An	81-year-old	woman	presented	with	a	pelvic	mass.	The	computed	tomography	scan	showed	a	non-calcified	solid	cystic	mass,	10.4x11.5x6.7	centimeters.	She	underwent	total	abdominal	hysterectomy	with	bilateral	salpingo-oophorectomy,	omentectomy	and	ascitic	fluid	for	cytology.	Histopathology	reported	poorly	differentiated	thyroid	carcinoma	arising	in	struma	ovarii.	Currently	she	has	been	free	of	disease	for	5	months	from	the	date	of	diagnosis.		Keywords:	Poorly	differentiated	thyroid	carcinoma,	struma	ovariiSiriraj Med J 2016;68:109-113E-journal: http://www.sirirajmedj.com/ojsCorrespondence to: Pisutt SrichaikulE-mail: pisutt.srichaikul@gmail.comReceived 2 June 2015Revised 24 August 2015Accepted 2 September 2015the	 rare	nature	of	 this	condition,	no	consensus	 exists	on	the	surgical	and	post-surgical	treatment of	patients	with	 thyroid	 carcinoma	arising	 in	struma	ovarii.	We	report	a	 rare	case	of	poorly	differentiated	thyroid	carcinoma	arising	in	struma	ovarii	and	 review	 the	 literature	on	 the	general	agreement	for	diagnosis	and	treatment	of	 these	cancers.CASE REPORT	 	 An	81-year-old,	Thai	woman	presented	with	a	large	pelvic	mass	and	an	intermittent	dull	pain	in	her	pelvis	for	one	month.	Her	past	medical	history	included	well-controlled	hypertension	and	dyslipidemia.	She	had	no	family	history	of	ovarian	and	endocrine	 tumors.	The	pelvic	examination	and	the	computed	tomography	(CT)	scan	showed	a	non-calcified	solid-	cystic	mass,	10.4x11.5	x6.7	centimeters,	arising	possibly	from	the	right	ovary	(Fig	1).	A	small	well-defined	 thin	wall	cyst	of	110left	ovary,	2.7x1.8	centimeters,	was	also	noted.	There	was	no	ascitic	fluid,	organomegaly	and	intra-abdominal	 lymphadenopathy.	The	 tumor	 markers	including	cancer	antigen	(CA)	125	and	CA	19-9	were	unremarkable	(CA125=	38.38	U/mL, CA19-9=27.75	U/mL).	Her	gynecologic	operation	was	postponed	for	3	months	because	of	cerebral	infarction.	She	had	 a	 right	 carotid	 bulb	with	 severe	luminal	stenosis	and	calcified	plaque	which	extended	to	the	right	internal	carotid	artery.	She	underwent	right	carotid	endarterectomy	which	was	uneventful.	Her	abdominal	pain	was	not	worse	and	the	follow-up	of	ultrasonography	showed	the	same	findings	as	Fig	2.	 	 Finally,	 the	 patient	 underwent	 a	 total	abdominal	hysterectomy	(TAH)	with	bilateral	salpingo-oophorectomy	(BSO),	omentectomy	and	ascitic	fluid	for	cytology.	There	was	1,600	mL	of	straw	colored	ascitic	fluid.	Her	right	ovary	had	become	a	solid-cystic	mass.	There	was	a	ruptured	site	on	 its	external	surface.	Her	uterus	and	 left	ovary	were	unremarkable.	The	tumor	was	totally	removed.	The	pathologic	report	revealed	poorly	differentiated	thyroid	carcinoma	arising	in	struma	ovarii.	The	ascitic	fluid	for	cytology	showed	some	atypical	cells.Gross pathology	 	 The	size	of	right	ovarian	mass	was	10x8.5x7 centimeters.	Its	external	surface	was	smooth	and	glistening	with	focal	nodular	area	(Fig	3A).	Cut	surfaces	showed	non-homogeneous	solid-cystic	lesion.	The	solid	component	was	brown-tan,	and gelatinous	appearance	with	multiple	foci	of	hemor- rhage	and	necrosis	and	it	occupied	approximately	70%	of	 the	 total	mass	 (Fig	3B,	3C).	The	 inner	cystic	surface	was	smooth	and	glistening	without	papillary	growth.	Few	small	foci	of	solid	gray-white	nodules	(varing	from	1	to	2.5	centimeters)	were	identified	(Fig	3D).Histopathology	 	 The	tumor	was	composed	of	mature	thyroid tissue	which	was	varying	in	size	of	thyroid	follicles and	containing	colloid	material.	Thyroglobulin	and	TTF-1	immunohistochemistry	were	identified	in	 the	epithelial	cell.	 In	areas	corresponding	 to	solid	gray-white	nodule,	the	section	demonstrated	Fig 1.	CT	imaging	showed	a	non-calcified	solid-cystic	mass	in	pelvic	cavity	(arrow).A BFig 2. A:	Ultrasonography	revealed	the	same	size	of	solid	cystic	mass	(arrow).	B:	Doppler	ultrasonography	showed	a	 low	vascular	flow	(RI=0.66).ACBDFig 3. A,	B	and	C,	Gross	photography	of	the	specimen	showing	non-homogeneous	 solid-cystic	 lesion	with	brown	 tan	gelatinous	 appearance,	 hemorrhage	 and	 necrosis.	D,	A	focus	of	solid	gray	with	nodule.a	well-defined	solid	nest	of	tumor	cells	surrounded	by	 thin	fibrovascular	 septa	 (insular	histologic	pattern)	(Fig	4A).	Tumor	cells	were	uniform	and	contained	round	vesicular	nuclei	with	indistinct	nucleoli,	mitotic	figure	presented	approximately	Siriraj Med J, Volume 68, Number 2, March-April 2016 1118/10	HPF	(Fig	4B),	and	obvious	vascular	invasion and	necrosis	was	present	(Fig	4C,	4D).	Immuno-histochemistry	demonstrated	 that	 these	 tumor	cells	were	diffusely	strongly	reactive	with	TTF-1,	Thyroglobulin	and	CK7,	but	they	did	not	mark	with	CK20,	CDX-2,	Calcitonin,	Synaptophysin,	Chromogranin	A,	Galectin3,	CK19,	or	HBME1.	Based	on	these	findings	together	with	Turin	pro-posal	this	tumor	was	fulfilling	the	criteria	diag-nostic	of	poorly	differentiated	thyroid	carcinoma	arising	in	struma	ovaii.	 	 The	consulting	Head-Neck	surgeon	and	radiotherapist	 agreed	 to	 treat	 this	patient	 as	 a	thyroid	carcinoma	 rather	 than	ovarian	cancer.	Her	 thyroid	 function	 test	was	normal.	 Imaging	scan	revealed	no	residual	tumor.	Therefore,	we	decided	to	treat	her	with	thyroxine	therapy.	Cur-rently	she	has	been	free	of	disease	for	5	months	from	the	diagnosis.	DISCUSSION	 	 Thyroid	carcinoma	arising	in	struma	ovarii is	a	rare	disease.	The	age	of	presentation	is	usually	from	the	5th	to	6th	decade	of	life3.	The	symptoms	and	signs	include	a	pelvic	mass	(45%),	abdominal pain	 (40%),	 ascites	 (17%)	 in	which	 the	 fluid	rarely	contained	 tumor	cells,	menstrual	 irregu-larities	(9%),	hyperthyroidism	(5-8%)	and	deep	vein	thrombosis	(4%).	CA125	may	be	elevated	as	 found	 in	germ	cell	 tumors.	The	diagnosis	 is	usually	made	 post-operatively.	 There	 are	 no	imaging	specific	characteristics	for	this	 tumor4. Ultrasonography	usually	shows	a	heterogeneous	solid-cystic	mass.	The	Doppler	imaging	reveals	abundant	 low	resistance	blood	flow	within	 the	highly	vascular	central	portion	of	the	tumor.	CT	imaging	depicts	a	multiloculated	cystic	mass	with	different	densities	among	 the	 locules	and	solid	portion.	Calcification	may	be	 found.	Magnetic	resonance	 imaging	finds	multiloculated	cystic	mass	with	variable	signal	intensity	(stained	glass	appearance)	and	solid	component.	 	 Primary	thyroid	carcinoma	with	ovarian	metastasis	can	be	differentiated	by	clinical	exami-nation	and	thyroid	ultrasonography.	In	the	case	of	bilateral	ovarian	masses	without	teratomatous	features,	these	favor	ovarian	metastasis.	The	other	differential	diagnosis	of	malignancy	arising	 in	struma	ovarii	includes	strumal	carcinoid,	Brenner	tumor,	granulosa	cell	tumor	and	papillary	serous	cystadenoma	or	cystadenocarcinoma5.	The	 im-munohistochemistry,	such	as	thyroglobulin	TTF-1	inhibin	WT1	and	CA	125,	will	aid	to	differentiate	these	tumors.	 	 Devaney	et	al,6	proposed	that	the	patho-logic	diagnosis	of	 thyroid	carcinoma	arising	 in	struma	ovarii	should	adhere	to	the	same	criteria	used	for	primary	thyroid	cancer.	These	include	papillary	carcinoma,	follicular	carcinoma,	poorly	differentiated	carcinoma	and	undifferentiated	(anaplastic)	carcinoma.	Papillary	carcinoma	 is	the	most	common	form	(44%),	followed	by	folli-cular	carcinoma	(30%)	and	follicular	variant	of	papillary	carcinoma	(26%).	Recently,	Roth	et	al,7 described	an	extraovarian	dissemination	of	thyroid	elements	and	histological	 resemblance	 to	non-neoplastic	thyroid	tissue	as	highly	differentiated	follicular	carcinoma	of	ovarian	origin	(HDFCO).	It	has	a	very	good	prognosis.Treatment and management of thyroid carci-noma arising in struma ovarii	 	 The	paucity	of	published	data	makes	 it	difficult	 to	find	 the	best	 treatment	modality	 for	ACBDFig 4. A,	Histologic	section	showed	both	classic	struma	ovarii	 and	 a	well-defined	 solid	nest	 of	 tumor	 cells	 surrounded	by	thin	fibrovascular	septa	(insular	histo-logic	pattern).	B,	Insular	histologic	growth	pattern	with	frequent	mitotic	figure	 (circle).	C,	Obvious	vascular	invasion	(arrow).	D,	Tumor	necrosis	(arrow	head).112these	tumors.	For	women	who	wish	to	preserve	 fertility,	conservative	surgery	(unilateral	oopho-rectomy)	should	be	considered	if	there	is	a	uni- lateral	tumor	without	evidence	of	capsular	invasion or	gross	metastasis.	In	the	case	presenting	with	gross	metastasis,	capsular	 invasion	or	an	 intra-operative	frozen	section	confirming	thyroid	car-cinoma	arising	 in	 struma	ovarii	 should	have	complete	surgical	staging	for	ovarian	cancer2.	 	 DeSimone	et	al,8	studied	the	postoperative	management	for	patients	with	malignant	struma	ovarii.	In	his	review	of	24	cases,	there	were	8	recur- rences	and	all	occurred	 in	 the	patients	without	postoperative	 treatment.	Hence,	 they	suggested	that	 treatment	with	 thyroidectomy	and	 Iodine	131	(I131)	should	be	considered	as	the	first	line	of	management.		 	 Yassa	et	al,9	classified	the	patients	accor-ding	to	the	risk	stratification.	In	low	risk	patients	with	thyroid	carcinoma	of	less	than	2	centimeters	in	 size,	 no	worrisome	histologic	 features	 and	 pelvic	 imaging	studies	showed	no	evidence	of	extraovarian	disease	 should	 receive	 thyroxine	therapy	to	suppress	thyroid	stimulating	hormone	secretion	and	periodic	total	body	scintiscanning	with	I131	and	serum	thyroglobulin.	Patients	with	 serum	 thyroglobulin	over	10	ng/mL	are	more	 likely	to	have	recurrence,	based	on	primary	thyroid	carcinoma10.	I131	diagnostic	whole	body	scanning	is	recommended.	Conversely,	patients	with	more	than	2	centimeters	in	size	of	thyroid	carcinoma,	aggressive	histologic	characters	or	extraovarian	disease	should	be	considered	as	high	risk	group.	Near-total	thyroidectomy	with	radioactive	iodine	therapy	is	recommended.		 	 Makani	 et	 al,2	 reported	 an	 average	 of	 4	years	for	detection	of	recurrence.	They	recom- mended	 at	 least	 10	 years	 for	 follow-up.	 I131 radioablation,	 external	 beam	 radiotherapy,	 chemotherapy	and	 thyroid	suppression	 therapy	have	been	used	 for	 treatment	 of	 recurrent	 or	metastatic	disease2,5.	 	 In	our	case,	the	diagnosis	of	poorly	dif-ferentiated	thyroid	carcinoma	was	based	on	the	Turin	proposal	for	the	use	of	uniform	diagnostic	criteria11.	These	include	(1)	presence	of	a	solid/trabecular/insular	pattern	of	growth,	(2)	absence	of	the	conventional	nuclear	features	of	papillary	carcinoma	and	(3)	presence	of	at	least	one	of	the	following	 features:	 convoluted	nuclei,	mitotic	activity	≥	3/10	high	power	fields	(HPF)	and	tumor necrosis.	 In	 primary	 thyroid	 carcinoma,	 the	 behavior	of	poorly	differentiated	carcinoma	lies	between	well	differentiated	and	undifferentiated	carcinoma.	Table	1	showed	 the	clinical	course	of	7	patients	with	poorly	differentiated	 thyroid	carcinoma	arising	in	struma	ovarii.	Because	of	the	small	number	of	patients	and	variety	of	surgeries	and	post-operative	treatments,	there	is	currently	no	agreement	for	treatment	of	these	patients.	Robboy	et	al,12	found	that	there	was	no	correlation	between	histologic	morphology	and	clinical	features.	Even	when	clinically	malignant,	 the	patient	 is	often	associated	with	 long	survival	as	evidenced	by	89%	10-year	and	84%	25-year	survival.	Since	our	patient	had	a	 recent	 stroke,	we	decided	 to	Reference Age, yrs stage Treatment of Follow-up   extraovarian diseaseWerth13,	1928	 36	 III	 Radiation		 AW	6	monthsGonzalez-Angulo14,	1963	 60	 I	 None	 NAZakhem15,	1990	 52	 IA	 NA	 NED	1.7	yrsZakhem15,	1990	 30	 IA	 NA	 NED	2.7	yrsRoth16,	2008		 70	 II	 Chemo,	RAI	 DOD	3	yrsGarg17,	2009	 40	 IA	 None		 NED	1.1	yrsGarg17,	2009	 47	 IA	 None		 NED	5	yrsTABLE 1. Cases	of	poorly	differentiated	thyroid	carcinoma	arising	in	struma	ovarii.AW,	alive	and	well;	DOD,	dead	of	disease;	NA,	not	available;	NED,	no	evidence	of	disease;	RAI,	radioactive	iodine	therapy	following	thyroidectomySiriraj Med J, Volume 68, Number 2, March-April 2016 113postpone	the	operation	for	3	months	and	undergo	the	shortest	time	for	surgery	to	remove	the	tumor	(TAH,	BSO,	omentectomy	and	ascitic	fluid	for	cytology).	In	the	multidisciplinary	meeting,	it	was	decided	that	because	there	was	no	residual	tumor	postoperatively,	her	associated	disease	was	quite	severe	and	she	is	81	years	old,	the	patient	should	be	followed	up	with	thyroxine	therapy.		 	 In	conclusion,	based	on	case	series	and	case	report,	treatment	modalities	of	thyroid	carci-noma	arising	in	struma	ovarii	depend	on	the	stage	of	disease	and	the	risk	stratification.	Limitation	for	the	recommendation	of	treatment	was	founddue	to	the	rarity	of	the	disease.	A	prospective	trial	is	not	likely	feasible.REFERENCES 1.		 Zhang	X,	Axiotis	C.	Thyroid	type	carcinoma	of	struma	 	 ovarii.	Arch	Patho	Lab	Med	2010;134:786-91.2.		 Makani	M,	Kim	W,	Gaba	AR.	Struma	ovarii	with	a	focus	 	 of	papillary	thyroid	cancer:	a	case	report	and	review	litera- 	 ture.	Gynecol	Oncol	2004;94:835-9.3.		 Salman	WD,	Singh	M,	Twaij	Z.	A	case	of	papillary	thyroid	 	 carcinoma	in	struma	ovarii	and	review	of	the	literature.	 	 Patholog	Res	Int	2010;2:352476.	4.		 Leite	I,	Cunha	TM,	Figueiredo	JP,	Felix	A.	Papillary	carci- 	 noma	arising	in	struma	ovarii	versus	ovarian	metastasis	 	 from	primary	thyroid	carcinoma:	a	case	report	and	review	 	 of	the	literature.	J	Radiol	Case	Rep	2013;7:24-33.5.		 Roth	LM,	 	Talerman	A.	The	enigma	of	struma	ovarii.	 	 Pathology	2007;39:139-46.6.		 Devaney	K,	Snyder	R,	Norris	H,	Tavassoli	F.	Proliferative	 	 and	histologically	malignant	 struma	ovarii:	 a	clinico- 	 pathologic	study	of	54	cases.	Int	J	Gynecol	Pathol	1993; 	 12:333-43.7.		 Roth	LM,	Karseladze	AI.	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Poorly Differentiated Thyroid Carcinoma Arising in Struma Ovarii: Case Report and Review of the Literature

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