Extraovarian fibroma–thecoma tumors in context
Fibromas and thecomas belong to the sex cord–stromal tumor group and are usually benign and ovarian in origin. Fibromas represent about 4% of ovarian tumors, while thecomas account for roughly 0.5%–1%. When these tumors arise outside the ovary, they become exceptionally rare and clinically problematic. Their extraovarian location blurs an already difficult diagnostic line, because they may present with findings typically linked to ovarian cancer, including ascites, elevated CA-125 levels, and occasionally pleural effusion.
These overlapping features often place patients on an oncologic diagnostic pathway, even though the underlying disease is benign. As the authors note, extraovarian sex cord–stromal tumors are “frequently misdiagnosed as ovarian cancers because of overlapping clinical and radiological features.”
Clinical presentation that raises concern for malignancy
Both patients described in the report were postmenopausal and presented with large abdominal masses. Preoperative imaging suggested ovarian malignancy in each case. In one patient, markedly elevated CA-125, ascites, and pleural effusion closely resembled Meigs syndrome. In the other, a solid mass with ascites prompted similar concern.
Intraoperative findings were unexpected. One tumor was located in the broad ligament, while the other was adherent to the omentum and intestines, with no clear ovarian involvement. These findings supported an extraovarian origin and highlighted how tumor location may only become evident during surgery.
Histologic features supporting fibrothecoma
Microscopic examination in both cases revealed patterns typical of fibroma–thecoma spectrum tumors. The lesions were composed of spindle to oval cells arranged in fascicular or storiform patterns, with focal areas showing lipid-rich, theca-like cells. Mitotic activity was low, and no features of overt malignancy were identified.
Special stains and careful morphologic assessment helped reinforce the diagnosis, particularly in distinguishing these tumors from other spindle cell lesions that may arise in the pelvis or abdomen.
Immunohistochemistry to exclude mimics
Immunohistochemical analysis played a central role in narrowing the diagnosis. The tumors expressed markers consistent with sex cord–stromal differentiation, including vimentin, WT1, progesterone receptor, and variable estrogen receptor, inhibin, calretinin, and CD56.
Equally important was the absence of staining for epithelial, melanocytic, and gastrointestinal stromal tumor markers. This negative profile was essential in excluding malignancies that can involve or arise near the bowel and omentum, particularly in the second case.
What the literature review reveals about rarity
To place their findings in context, the authors reviewed published reports of extraovarian fibroma–thecoma tumors. Only a small number of well-documented cases were identified. Most arose in the broad ligament or pelvic cavity, and many were associated with ascites and elevated CA-125 levels—features that contributed to preoperative suspicion of cancer.
The review underscores how limited collective experience remains and why these tumors are still underrecognized in routine clinical practice.
Implications for diagnosis and surgical management
The key message of the paper is practical. Elevated CA-125 and fluid accumulation do not necessarily indicate malignancy, even in postmenopausal patients with large pelvic or abdominal masses. Awareness of extraovarian fibrothecoma can prompt more cautious interpretation of preoperative findings.
The authors emphasize that accurate pathological evaluation is critical to avoiding unnecessary radical surgery. Recognizing this rare benign entity allows clinicians and pathologists to align surgical extent with true biological behavior, improving patient outcomes while reducing overtreatment.
The translation of the preceding English text in Bosnian:
Ekstraovarijalni fibrom–tekoma tumori u kontekstu
Fibromi i tekomi pripadaju grupi tumora polnih vrpci–strome i obično su benigni i ovarijalnog porijekla. Fibromi čine oko 4% tumora jajnika, dok tekomi čine približno 0,5%–1%. Kada se ovi tumori jave izvan jajnika, postaju izuzetno rijetki i klinički problematični. Njihova ekstraovarijalna lokalizacija dodatno zamagljuje već složenu dijagnostičku granicu, jer se mogu prezentirati nalazima koji su tipično povezani s karcinomom jajnika, uključujući ascites, povišene vrijednosti CA-125 i povremeno pleuralni izljev.
Ove preklapajuće karakteristike često usmjeravaju pacijente na onkološki dijagnostički put, iako je osnovna bolest benigna. Kako autori navode, ekstraovarijalni tumori polnih vrpci–strome su „često pogrešno dijagnosticirani kao karcinomi jajnika zbog preklapajućih kliničkih i radioloških karakteristika“.
Klinička prezentacija koja pobuđuje sumnju na malignitet
Obje pacijentice opisane u izvještaju bile su u postmenopauzi i prezentirale su se velikim abdominalnim masama. Preoperativno snimanje je u oba slučaja sugeriralo malignitet jajnika. Kod jedne pacijentice, izrazito povišen CA-125, ascites i pleuralni izljev su u velikoj mjeri podsjećali na Meigsov sindrom. Kod druge je solidna masa uz ascites izazvala sličnu zabrinutost.
Intraoperativni nalazi bili su neočekivani. Jedan tumor je bio smješten u širokom ligamentu, dok je drugi bio adherentan uz omentum i crijeva, bez jasnog zahvatanja jajnika. Ovi nalazi su podržali ekstraovarijalno porijeklo i naglasili kako lokalizacija tumora često postaje očigledna tek tokom operacije.
Histološke karakteristike koje podržavaju dijagnozu fibrothekome
Mikroskopski pregled u oba slučaja otkrio je obrasce tipične za tumore iz spektra fibrom–tekoma. Lezije su bile sastavljene od vretenastih do ovalnih ćelija raspoređenih u fascikularne ili storiformne obrasce, s fokalnim područjima bogatim lipidima, nalik tekalnim ćelijama. Mitotska aktivnost je bila niska, a nisu uočene karakteristike otvorenog maligniteta.
Posebna bojenja i pažljiva morfološka procjena dodatno su potvrdili dijagnozu, naročito u razlikovanju ovih tumora od drugih vretenastih ćelijskih lezija koje se mogu javiti u zdjelici ili abdomenu.
Imunohistohemija u isključivanju diferencijalnih dijagnoza
Imunohistohemijska analiza imala je centralnu ulogu u sužavanju dijagnoze. Tumori su eksprimirali markere u skladu s diferencijacijom polnih vrpci–strome, uključujući vimentin, WT1, progesteronski receptor i varijabilno estrogenski receptor, inhibin, kalretinin i CD56.
Jednako važno bila je odsutnost bojenja na epitelne, melanocitne i markere gastrointestinalnih stromalnih tumora. Ovaj negativni profil bio je ključan za isključivanje maligniteta koji mogu zahvatiti ili nastati u blizini crijeva i omentuma, naročito u drugom slučaju.
Šta pregled literature otkriva o rijetkosti
Kako bi svoje nalaze stavili u kontekst, autori su pregledali objavljene izvještaje o ekstraovarijalnim fibrom–tekoma tumorima. Identificiran je samo mali broj dobro dokumentiranih slučajeva. Većina je nastala u širokom ligamentu ili zdjeličnoj šupljini, a mnogi su bili povezani s ascitesom i povišenim vrijednostima CA-125 — karakteristikama koje su doprinijele preoperativnoj sumnji na karcinom.
Ovaj pregled naglašava koliko je kolektivno iskustvo još uvijek ograničeno i zašto su ovi tumori i dalje nedovoljno prepoznati u rutinskoj kliničkoj praksi.
Implikacije za dijagnozu i hirurško zbrinjavanje
Ključna poruka rada je praktična. Povišen CA-125 i nakupljanje tečnosti ne ukazuju nužno na malignitet, čak ni kod postmenopauzalnih pacijentica s velikim zdjeličnim ili abdominalnim masama. Svijest o postojanju ekstraovarijalne fibrothekome može potaknuti opreznije tumačenje preoperativnih nalaza.
Autori naglašavaju da je precizna patološka evaluacija od presudne važnosti za izbjegavanje nepotrebne radikalne hirurgije. Prepoznavanje ovog rijetkog benignog entiteta omogućava kliničarima i patolozima da usklade obim hirurškog zahvata sa stvarnim biološkim ponašanjem tumora, čime se poboljšavaju ishodi liječenja i smanjuje pretjerano liječenje.
Reference:
Nejra Selak, Ivana Čerkez, Ermina Iljazović, Azra Sadiković, Maja Konrad Čustović, Jasminka Mustedanagić Mujanović, Edina Ahmetović Karić
Extraovarian fibrothecomas: Two case reports and comprehensive review of ovarian sex cord-stromal fibroma-thecoma tumors.
Biomol Biomed [Internet]. 2025 Aug. 18 [cited 2025 Dec. 15];26(3):509–524.
Available from: https://www.bjbms.org/ojs/index.php/bjbms/article/view/12816
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