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Thecoma

Theca Cell Tumor

Thecoma is a rare and almost invariably benign ovarian sex-cord stromal tumor that is predominantly diagnosed in postmenopausal women. In this patient group, an asymptomatic course is frequently observed. Hormone-secreting thecomas are, however, more frequent among younger women and associated with uterine bleeding, abdominal distension, and pain. Clinical findings and imaging studies are needed to make the diagnosis, but the exact tumor type is often determined postoperatively.


Presentation

The ovarian tumor thecoma belongs to a group of sex-cord stromal tumors, and the name stems from its lipid-rich stromal cells that are strikingly similar to theca cells located in the ovarian follicles [1] [2] [3]. Thecoma is virtually always a benign tumor (although rare malignant cases have been reported), and it is principally diagnosed in postmenopausal women [2] [4]. The clinical presentation depends on whether the tumor contains cells that produce steroid hormones (termed luteinized thecoma) or not (typical and rarely calcified thecoma) [2] [5]. Luteinized thecoma has been shown to appear at a slightly younger age and may produce estrogenic, virilizing, or mixed effects [2] [3] [5]. Non-secreting tumors rarely cause symptoms. Certain authors report that more than 60% of post-menopausal women are asymptomatic [6]. Tumors that synthesize estrogen, on the other hand, produce symptoms such as uterine bleeding (and menstrual irregularities in younger women), abdominal pain and/or distension, whereas an abdominal mass might be identified during a physical examination [1] [2]. In addition, between 20-50% of cases suffer from concomitant endometrial carcinoma [2] [5] [6]. Although they can present with worrying symptoms, the clinical course of thecoma is benign and proper surgical treatment results in a cure [6].

Severe Pain
  • Torsion, infarction or haemorrhage: Cause severe pain. Torsion may be intermittent, presenting with intermittent episodes of severe pain.[patient.info]
Severe Abdominal Pain
  • Torsion or rupture may lead to severe abdominal pain and fever. Dyspareunia. Swollen abdomen, with palpable mass arising out of the pelvis, which is dull to percussion and does not disappear if the bladder is emptied.[patient.info]
Hirsutism
  • Although laser treatment helped with her hirsutism, her other virilizing symptoms (deepening of voice, clitoromegaly) did not improve postoperatively.[ncbi.nlm.nih.gov]
  • In contrast to other virilizing ovarian tumors, which present as rapidly progressive hirsutism, ovarian thecoma (like hilus cell tumors of ovary) can present as slowly progressive hirsutism. 1. Carmina E, Rosato F, Jannì A, Rizzo M, Longo RA.[ijem.in]
  • A 49-year-old woman presented with rapidly progressing hirsutism, receding hairline, male-pattern baldness and deepening of voice, which had developed over the past 2 years.[ncbi.nlm.nih.gov]
  • A 29-yr-old woman with previously normal reproductive function, including prior fertility, was evaluated for amenorrhea and hirsutism.[ncbi.nlm.nih.gov]
  • A 31-yr-old hirsute female with oligoamenorrhea since menarche had markedly elevated peripheral plasma testosterone (T) concentrations of 250-255 ng/100 ml (normal 20-60 ng/100 ml), which lacked a diurnal rhythm, were not suppressed by dexamethasone,[ncbi.nlm.nih.gov]
Receding Hairline
  • A 49-year-old woman presented with rapidly progressing hirsutism, receding hairline, male-pattern baldness and deepening of voice, which had developed over the past 2 years.[ncbi.nlm.nih.gov]
Primary Amenorrhea
  • These tumors had presented as primary amenorrhea, secondary amenorrhea, hirsutism, menstrual irregularity or pressure effects. Ovarian thecoma with virilizing manifestations were reported in both post-menopausal and premenopausal age groups.[ijem.in]

Workup

The presence of abnormal uterine bleeding or menstrual abnormalities should prompt the physician to perform a meticulous clinical and imaging workup. Firstly, a detailed patient history including the onset of symptoms and their progression must be noted, followed by a complete physical examination, with an emphasis on abdominal and pelvic inspection/palpation. As soon as initial suspicion is raised, imaging studies should be employed. Abdominal ultrasonography (US) is a highly useful initial method in the evaluation of abdominal pathologies. The presence of a unilateral adnexal hypoechoic mass possessing clear borders and a weak vascular signal on Doppler imaging are hallmarks of a thecoma [6]. To confirm the diagnosis, however, more advanced studies - computed tomography (CT) and magnetic resonance imaging (MRI), are often necessary. CT shows an isodense or hypodense tumor, whereas isointense signaling on T1- weighted images and either hypointense or isointense signaling T2- weighted images are typical findings (although some authors claim that even hyperintense signals can be encountered on T2, depending on the amount of surrounding edema) [3] [6] [7] [8] [9]. In addition to imaging procedures, the use of ovarian serum tumor marker (CA 125) in the diagnostic workup might also be recommended, as it is frequently elevated in the presence of a neoplastic process in the ovaries [1] [10].

Treatment

  • Diagnosis and treatment of ovarian thecoma occurring during pregnancy are discussed with relevant references.[ncbi.nlm.nih.gov]
  • Luteinized thecoma of the ovary associated with sclerosing peritonitis is a rare pathologic condition without a standard strategy of treatment. We present the case of an ovarian luteinizing sclerosing thecoma in a 39-year-old woman.[ncbi.nlm.nih.gov]
  • Treatment should be aimed at relief of bowel obstruction symptoms, preferably with conservative methods.[ncbi.nlm.nih.gov]
  • Although laser treatment helped with her hirsutism, her other virilizing symptoms (deepening of voice, clitoromegaly) did not improve postoperatively.[ncbi.nlm.nih.gov]
  • Luteinized thecoma of the ovary associated with sclerosing peritonitis is a rare tumor that has no standard definitive treatment regimen. A 25 year-old patient diagnosed with luteinized thecoma and sclerosing peritonitis in the omentum.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis With early detection and treatment the prognosis for thecoma is quite good.[knowcancer.com]
  • Treatment and prognosis Thecomas are almost always benign and have low malignant potential, although there have been isolated reports of malignant thecomas. fibroma low signal on T2 given the fibroid content less enhancement a dult granulosa cell tumour[radiopaedia.org]
  • Fox H, Agrawal K, Laugley F (1975) A clinicopathologic study of 92 cases of granulosa cell tumour of the ovary with special reference to the factors affecting prognosis. Cancer 35:231–241 PubMed CrossRef Google Scholar 5.[link.springer.com]
  • Prognosis and predictive factors About 30% of cases are clinically malignant and have an extraovarian spread of tumor at the time of operation.[emedicine.medscape.com]
  • In addition to its pathology and genetics, each lesion is described with its epidemiology, etiology, clinical features, as well as prognosis and predictive factors. This book is in the series commonly referred to as the "Blue Book" series.[books.google.com]

Etiology

  • Luteinizing thecoma with sclerosing peritonitis (LTSP) is a rare ovarian tumor of unclear etiology and pathogenesis.[ncbi.nlm.nih.gov]
  • Etiology Thecoma-fibroma tumors are a closely related group of benign tumors that arise from ovarian stroma and are often difficult for the imager and even pathologist to distinguish.[clinicalgate.com]
  • In addition to its pathology and genetics, each lesion is described with its epidemiology, etiology, clinical features, as well as prognosis and predictive factors. This book is in the series commonly referred to as the "Blue Book" series.[books.google.com]
  • Wilms' tumor a rapidly developing malignant mixed tumor of the kidneys, made up of embryonal elements, occurring chiefly in children before the seventh year; a genetic component is suspected in its etiology.[medical-dictionary.thefreedictionary.com]

Epidemiology

  • In addition to its pathology and genetics, each lesion is described with its epidemiology, etiology, clinical features, as well as prognosis and predictive factors. This book is in the series commonly referred to as the "Blue Book" series.[books.google.com]
  • Sertoli–Leydig cell tumor group Epidemiology Sertoli-Leydig cell tumors are uncommon benign tumors, accounting for less than 0.5% of all ovarian tumors.[emedicine.medscape.com]
  • Epidemiology Benign ovarian tumours occur in 30% of females with regular menses (eg, luteal cysts as incidental findings on pelvic scans) and 50% of females with irregular menses.[patient.info]
  • Epidemiology The frequency of sex cord-stromal tumours is similar throughout the world. There does not appear to a racial predisposition, in contrast to epithelial ovarian cancers.[atlasgeneticsoncology.org]
Sex distribution
Age distribution

Prevention

  • Awareness of this newly described condition, which may be more common than previously thought, may help prevent further misdiagnosis.[ncbi.nlm.nih.gov]
  • […] removal of pelvic and para-aortic lymph nodes and omentum Chemotherapy combination of platinum (i.e. carboplatin) and non-platinum (i.e. paclitaxel) regimen Radiation reserved for palliation or localized persistent disease after chemotherapy Prognosis, Prevention[medbullets.com]
  • Prevention of intractable partial seizures by intermittent vagal stimulation in humans:preliminary results. Epilepsia 1990;31(suppl 2):S40-43 26. Ben-Menachem E., Manon-Espaillat R., Ristanovic R. et al.[revistamedicina.net]

References

Article

  1. Zhang Z, Wu Y, Gao J. CT diagnosis in the thecoma-fibroma group of the ovarian stromal tumors. Cell Biochem Biophys. 2015;71:937-43.
  2. Tanaka YO, Tsunoda H, Kitagawa Y et-al. Functioning ovarian tumors: direct and indirect findings at MR imaging. Radiographics. 2004;24 Suppl: S147-66.
  3. Shanbhogue AK, Shanbhogue DK, Prasad SR, et al. Clinical syndromes associated with ovarian neoplasms: a comprehensive review. Radiographics. 2010;30(4):903-919.
  4. Chen VW, Ruiz B, Killeen JL, et al. Pathology and classification of ovarian tumors. Cancer. 2003;97:2631-42.
  5. Kulkarni YS, Kakade AS, Singh BA. A rare case of ovarian thecoma in a postmenopausal woman. Int J Reprod Contracept Obstet Gynecol 2014;3:242-244.
  6. Chen H, Liu Y, Shen L, et al. Ovarian thecoma-fibroma groups: clinical and sonographic features with pathological comparison. J Ovarian Res. 2016;9:81.
  7. Jung SE, Lee JM, Rha SE, Byun JY, et al. CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. Radiographics. 2002; 22:1305-1325.
  8. Troiano RN, Lazzarini KM, Scoutt LM, et al. Fibroma and fibrothecoma of the ovary: MR imaging findings. Radiology. 1997; 204:795-798
  9. Jeong YY, Outwater EK, Kang HK. Imaging evaluation of ovarian masses. Radiographics. 2000; 20:1445-1470.
  10. Yazdani S, Alijanpoor A, Sharbatdaran M, et al. Meigs’ syndrome with elevated serum CA125 in a case of ovarian fibroma /thecoma. Caspian J Intern Med. 2014;5(1):43-45.

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Last updated: 2017-08-09 18:18