Ovarian Fibroma Research Articles

Pseudo–Meigs’ syndrome secondary to bilateral ovarian endometrioid carcinomas

w x Meigs’ syndrome 1 was first reported in a patient with ovarian fibroma accompanied by ascites and pleural effusion, which resolved after resection of the tumor. However, some cases of the syndrome were reported later in patients with ovarian tumors other than fibroma, or even tumors of uterus and fallopian tubes, which are w x then referred to as pseudo-Meigs’ syndrome 2]4 . We report a case of pseudo-Meigs’ syndrome, which was associated with bilateral ovarian carcinomas. To our knowledge, this is the second case of pseudo-Meigs’ syndrome associated with ovarian carcinoma. A 46-year-old woman presented with a history of right hydrothorax for 3 months and right ovarian mass for 5 months. Before being admitted to our hospital, she was treated as having pulmonary

MR imaging and CT of the female pelvis: radiologic-pathologic correlation.

For imaging of the female pelvis, transvaginal ultrasound is the method of choice. Magnetic resonance imaging and CT provide important additional information in various disorders. Magnetic resonance imaging is superior to CT in diagnosing benign and malignant disorders of the uterus. The same holds for the characterization of adnexal masses, where MR imaging reliably differentiates dermoids, ovarian fibromas, and most endometriomas. Differentiation of other benign and malignant ovarian tumors by CT and MR imaging is based on identical morphological criteria; no superiority of MR imaging over CT has been established. Computed tomography is still the preferred imaging modality for staging ovarian cancer. A thorough knowledge of the pathomorphological changes associated with the different disorders of the female pelvis not only helps to choose the proper imaging modality and examination protocol, but also improves image interpretation.

Ovarian smooth muscle metaplasia: an uncommon and possibly underrecognized entity.

Smooth muscle differentiation in the ovary is rare, and its histopathologic spectrum, including ovarian smooth muscle metaplasia (SMM), has not been well described. The clinicopathologic findings in 48 ovaries with SMM from 40 women are reported. The average age of women with ovarian SMM was 55.6 years (range, 34 to 86 years). Foci of SMM were semiquantitatively characterized as 1+ in 46% (1 to 3 foci), 2+ in 37% (4 to 6 foci), and 3+ in 17% (> 6 foci). SMM was bilateral in 8 (23%) of the 35 patients who had bilateral oophorectomies. SMM was intimately associated with another ovarian process in 28 (58%) cases, including ovarian cysts (11), endometriosis (3), granulosa cell tumors (3), extensive stromal luteinization (1), ovarian fibroma (1), adhesions (1), and folliculogenesis (8). Ovaries with 2+ to 3+ SMM were associated with another ovarian lesion significantly more often than those with 1+ SMM (p < 0.01). Most women with ovarian SMM (86%) also had uterine leiomyomas. Significant endometrial pathology was present in 13 (37%) of 35 simultaneously removed uteri. In conclusion, SMM occurs most often in perimenopausal or postmenopausal women, most of whom also have uterine leiomyomas. Ovarian SMM is usually confined to a few microscopic fields, is bilateral in < 25% of patients, and is often associated with other ovarian lesions.

Meigs' syndrome with elevated CA125: case report and review of the literature

A 51-year-old woman presented with acute ascites and hydrothorax is reported. Pelvic ultrasound showed two pelvic solid masses and serum CA125 level was elevated (577 IU/ml). Pathology revealed bilateral ovarian fibromas. We present this case of Meigs' syndrome and discuss its diagnostic problems and a review of the literature.

Fortgeschrittener Unterbauchtumor mit Aszites in der Schwangerschaft bei Gorlin-Goltz-Syndrom

Bei einer 19jährigen Erstgravida mit Gorlin-Goltz-Syndrom, einer seltenen erblichen Erkrankung (1:57000), die gehäuft rnit benignen, selten auch mit malionen Ovarialtumoren einhergeht, wurde in der 8. SSW retrouterin ein solider, stark vaskularisierter Tumor von knapp 10 cm Durchmesser festgestellt. Sonographisch gesteuerte Stanzbiopsien in der 9. SSW ergaben fibromuskuläres Gewebe. In den folgenden Wochen zeigte der Tumor rasche Wachstumstendenz und führte ab der 26, SSW bei subjektivem Wohlbefinden zu massivem Aszites. Pleuraergüssen und einem rechtsseitigen Harnstau II.-III.°. Trotz des benignen Biopsiebefundes und einer Punktionszytologie des Aszites ohne Nachweis maligner Zellen bestand in Anbetracht der raschen Größenzunahme, der ausgeprägten Ergußbildung und exzessiv angestiegener CA 12-5 Werte über 4000 U/ml der dringende Verdacht auf ein Malignom. Unter Abwägung mütterlicher und kindlicher Risiken wurde nach medikamentöser Lungenreifung mit Betamethason in der 29. SSW der elektive Kaiserschnitt per Längsschnittlaparotomie vorgenommen. mit Darm und Uterus verwachsene, ca. 20 cm große Tumor operativ entfernt. Histologisch ergab sich ein zellreiches, wahrscheinlich vom rechten Ovar ausgehendes Leiomyom mit größeren fibromatösen Anteilen. Anhand des Falles wird das diadächtigen Unterbauchtumoren in der Schwangerschaft diskutiert.

The role of ultrasonography in the detection and management of adnexal masses during the second and third trimesters of pregnancy

OBJECTIVE: Our purpose was to determine the effect of routine second-trimester and third-trimester ultrasonographic examinations on the prevalence of detectable and operable adnexal disease. STUDY DESIGN: The study group consisted of 7996 pregnant women between 13.0 and 42.8 weeks’ gestation. The size and architectural pattern of any detectable adnexal masses were noted. RESULTS: A total of 328 of the 7996 (4.1%) women in the study group had 335 ultrasonographically detectable adnexal masses; 309 of the masses were unilocular or had a single thin septation and 26 were architecturally complex. Of the ovarian cysts 252 of 309 (81.6%) had a mean diameter < 3.0 cm; 60% of the 252 patients in this subgroup had serial ultrasonographic examinations; 43 of the unilocular cysts resolved, and 17 have persisted for up to 2 years. There is a statistically significant trend toward decreasing frequency of ovarian cysts with increasing gestational age (χ 2 for linear trend; P < .00001). Eighteen of the 7996 had an exploratory laparotomy (1 operation per 444 deliveries) during pregnancy or in the postpartum period. In addition, 1 patient had a paratubal cyst excised at the time of postpartum bilateral tubal ligation. Pathologically confirmed lesions included 8 benign cystic teratomas, 3 mucinous cyst adenomas, 2 paratubal cysts, 2 corpus lutea, 1 serous cystadenoma, 1 follicular cyst, 1 endometrioma, and 1 ovarian fibroma. CONCLUSION: Ovarian cysts are found in 4.1% of second-trimester and third-trimester obstetric ultrasonographic examinations. Most ultrasonographically detectable cysts are < 3.0 cm in diameter and usually resolve. The frequency of exploratory laparotomy for adnexal disease is not significantly different from that in reports before the widespread use of obstetric ultrasonography. (Am J Obstet Gynecol 1998;179:703-7.)

Ovarian fibromas and cystadenofibromas: MRI features of the fibrous component

Ovarian fibromas and cystadenofibromas are neoplasms that share a similar distinctive tissue component of dense fibrous tissue. We sought to describe the MRI features of these neoplasms and to determine if the fibrous component shows distinctive characteristics. Fourteen patients in whom MR images performed with multicoil and fast-spin-echo images and who subsequently underwent surgery for resection of ovarian fibromas or cystadenofibromas were identified from two institutions. Five patients had ovarian fibromas, and nine patients had fourteen cystadenofibromas. 1.5-T MR studies used T1-weighted spin echo and multiplanar T2-weighted fast-spin-echo images, with fat saturation gadolinium-enhanced fast multiplanar gradient-echo images in seven patients. Studies were reviewed for findings of low (approximately equal to skeletal muscle) signal intensity solid components on T2-weighted images, characteristics of gadolinium enhancement, and associated endometrial findings. Images were obtained ex vivo from three adnexal surgical specimens with an 8-cm field of view and correlated with histology. All five of the fibromas showed predominantly very low signal intensity, similar to skeletal muscle, on T2-weighted images. Two of five fibromas were in patients with endometrial polyps and increased amounts of fluid in the pelvis. Thirteen cystadenofibromas were multicystic masses with bands of very low signal intensity ranging from 2 to 20 mm in the wall of the mass, and one was predominantly solid fibrous tissue. Pathologic correlation with specimen images showed that the low signal intensity material was the subepithelial fibrous component of the cystadenofibromas. Fibrous components of ovarian fibromas and cystadenofibromas are demonstrable by MR as solid components representing fibrous tissue of very low signal intensity on T2-weighted images.

Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome

Nevoid basal cell carcinoma syndrome (NBCC; Gorlin syndrome), an autosomal dominant disorder linked to 9q22.3-q31, and caused by mutations in PTC, the human homologue of the Drosophila patched gene, comprises multiple basal cell carcinomas, keratocysts of the jaw, palmar/plantar pits, spine and rib anomalies and calcification of the falx cerebri. We reviewed the findings on 105 affected individuals examined at the NIH since 1985. The data included 48 males and 57 females ranging in age from 4 months to 87 years. Eighty percent of whites (71/90) and 38% (5/13) of African-Americans had at least one basal cell carcinoma (BCC), with the first tumor occurring at a mean age of 23 (median 20) years and 21 (median 20) years, respectively. Excluding individuals exposed to radiation therapy, the number of BCCs ranged from 1 to > 1,000 (median 8) and 1 to 3 (median 2), respectively, in the 2 groups. Jaw cysts occurred in 78/105 (74%) with the first tumor occurring in 80% by the age of 20 years. The number of total jaw cysts ranged from 1 to 28 (median 3). Palmar pits and plantar pits were seen in 87%. Ovarian fibromas were diagnosed by ultrasound in 9/52 (17%) at a mean age of 30 years. Medulloblastoma occurred in 4 patients at a mean age of 2.3 years. Three patients had cleft lip or palate. Physical findings include "coarse face" in 54%, relative macrocephaly in 50%, hypertelorism in 42%, frontal bossing in 27%, pectus deformity in 13%, and Sprengel deformity in 11%. Important radiological signs included calcification of the falx cerebri in 65%, of the tentorium cerebelli in 20%, bridged sella in 68%, bifid ribs in 26%, hemivertebrae in 15%, fusion of the vertebral bodies in 10%, and flame shaped lucencies of the phalanges, metacarpal, and carpal bones of the hands in 30%. Several traits previously considered components of the syndrome (including short fourth metacarpal, scoliosis, cervical ribs and spina bifida occulta) were not found to be significantly increased in the affected individuals. This study delineates the frequency of the clinical and radiological anomalies in NBCC in a large population of US patients and discusses guidelines for diagnosis and management.

Characterization of a YAC contig containing the NBCCS locus and a novel Kruppel-type zinc finger sequence on chromosome segment 9q22.3

Gorlin's syndrome or nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder characterized by a familial or hereditary predisposition to basal cell carcinomas (generally multiple and of early onset), odontogenic keratocysts (jaw cysts), palmar and plantar pits, a wide variety of developmental defects, as well as cancers such as medulloblastomas and ovarian fibromas. The gene for NBCCS has been mapped to human chromosome region 9q22.1-q31 by linkage analysis and by cytogenetic evidence of deletions in this region in patients with the syndrome. This is supported by loss of heterozygosity in tumors of polymorphic marker loci flanked by D9S197 and D9S180. We have utilized sequence tagged site (STS) mapping and somatic cell hybrid panel analysis to construct two overlapping yeast artificial chromosome (YAC) contigs spanning this region of the genome. We used the YAC contigs to identify a new zinc finger gene containing a highly informative microsatellite locus.

Benign fibromatous tumors of the testis and paratesticular region: a report of 9 cases with a proposed classification of fibromatous tumors and tumor-like lesions.

Benign intrascrotal fibrous proliferations are uncommon, with most arising from the paratesticular region and falling into the category of so-called fibrous pseudotumor. We describe two neoplastic forms of benign fibrous tumor of the testis and its adnexa: fibroma of gonadal stromal origin and fibroma of the testicular tunics. Three patients with gonadal stromal fibroma were 28, 33, and 35 years of age and presented with painless masses. The tumors were circumscribed, intratesticular, yellow-white or white lesions 0.9 to 4.0 cm in diameter and had microscopic features identical to those of the ovarian fibroma. Immunohistochemically, the tumor cells were strongly positive for vimentin (3/3 cases), focally positive for actin and desmin (2/3 cases) and negative for S-100, keratin, and CD34 (3/3 cases). Six fibromas of the testicular tunics arose in patients 22, 34, 60, 68, 70, and 74 years old and were also asymptomatic masses. Four of them were circumscribed, whorled, white masses arising from the tunica albuginea with variable areas of myxoid change; one was pedunculated and grew exclusively into the cavity of the tunica vaginalis, whereas the other three at least partially extended into the testis as well. The other two tumors were unattached to the tunica albuginea and presented as circumscribed, white-tan paratesticular masses, partially covered by tunica vaginalis. Microscopically, the tumors were slightly to moderately cellular, with bland spindle or stellate cells lying in a myxoid or collagenous stroma with prominent vessels. The two paratesticular tumors had features typical of solitary fibrous tumor. Immunohistochemically, the fibromas of the testicular tunics were negative for S-100, keratin, and desmin. Focal, weak reactivity for actin was present in one case. CD34 was positive in three cases; in the two tumors resembling solitary fibrous tumors it was strong, and in the other it was focal and was limited to the region just below the tunica vaginalis. Eight tumors were treated by radical orchiectomy and one by excision of the mass alone. The outcome was benign in the seven cases in which followup information is available.

The Nevoid Basal Cell Carcinoma Syndrome: Genetics and Mechanism of Carcinogenesis

The nevoid basal cell carcinoma syndrome is an autosomal dominant disorder that predisposes to basal cell carcinomas of the skin, ovarian fibroma, and medulloblas-toma. Unlike many other hereditary disorders associated with cancer, it features widespread birth defects. Laboratoiy studies of radiation sensitivity and chromosome instability over the past 20 years have generally yielded negative or inconclusive results. Screening for allelic loss in sporadic and hereditary basal cell carcinomas, hereditary ovarian fibromas, and sporadic medulloblastomas provided evidence for a tumor suppressor gene on chromosome 9q important in all three tumor types. Demonstration of a constitutional chromosome 9q deletion in an unusual patient with this syndrome and genetic linkage studies in large kindreds indicated that the nevoid basal cell carcinoma syndrome gene maps to the same location lost in tumors. These data indicate that tumors arise with homozygous inactivation of the gene and imply that it normally functions as a tumor suppressor. In contrast, hemizygous germline mutations lead to multiple congenital anomalies.

CT and MR Findings of Ovarian Fibroma

CT and MR Findings of Ovarian Fibroma

Consistent use of bloodsaving measures in major cranio-maxillofacial surgery

Gorlin's syndrome or naevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder that predisposes to basal cell carcinomas of the skin, ovarian fibromas, and medulloblastomas. This condition is due to mutations in the Patched (PTCH) gene which maps to chromosome 9q22 and acts as a tumour suppressor gene. Gorlin's syndrome is characterized by the development of multiple jaw keratocysts and/or basal carcinomas. There is a distinctive coarse facial appearance with macrocephaly, frontal bossing and prognathism. Most individuals have skeletal anomalies such as bifid ribs or wedge-shaped vertebrae. We present a case in which the patient presented with bilateral thumb hypoplasia. Various hand deformities have been reported in patients with Gorlin's syndrome including short metacarpals, cutaneous syndactyly of the second and third fingers, and pre- or post-axial polydactyly, but hypoplasia of the thumb has not been reported previously. These features of Gorlin's syndrome may be helpful diagnostically. The thumbs should be examined carefully in Gorlin's syndrome patients as minor degrees of hypoplasia are easy to miss. However, they still needs a specialist input to give the patient an optimum function of the thumb and the hand.

Nevoid Basal-Cell Carcinoma (Gorlin) Syndrome

Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by large numbers of basal cell cancers and epidermal cysts of the skin, odontogenic keratocysts of the jaws, palmoplantar pits, calcified durai folds, various neoplasms or hamartomas which include medulloblastoma, ovarian fibroma, lymphomesenteric cysts, fetal rhabdomyoma, etc., and various stigmata of maldevelopment (rib and vertebral anomalies, cortical defects of bone, cleft lip and/or palate, etc.).

Ovarian fibroma in a prepubertal girl

Ovarian fibroma rarely occurs in prepubertal girls. However, we found such an asymptomatic, large, uncalcified pelvic mass in an otherwise healthy 12-year-old girl. The plain films (radiographs), US and CT findings are presented.

Characterization of human ovarian epithelial tumors (ex vivo) by proton magnetic resonance spectroscopy.

Proton magnetic resonance spectroscopy (1H MRS) offers an alternative investigational modality that will assist current pathologic techniques in the diagnosis of human ovarian epithelial tumors. Histologically normal human ovarian tissue (n = 12) was compared with ovarian benign fibromas (n = 3) and surface epithelial-stromal tumors (benign, n = 18; proliferating, n = 9; frankly malignant, n = 30) ex vivo by 1H MRS. The distinction between carcinomatous and benign or normal tissue (P<0.0001; Student's t-test) was made on one-dimensional (1-D) 1H MR spectra utilizing differences in resonance intensities of cellular lipid, creatine/phosphocreatine and lysine. The sensitivity and specificity of the test were 87% and 91%, respectively. Two-dimensional (2-D) MRS of carcinomatous biopsies showed multiple crosspeaks attributable to cell-surface fucosylation that correlated with tumor grade and loss of cellular differentiation. The multiple fucose crosspeaks were absent in spectra from normal ovary and benign tumors. The distinction between carcinomatous and normal or benign tissue based on MR-visible fucosylation gave a sensitivity and specificity of 88% and 97%, respectively. Proliferating tumors exhibited a range of cell-surface fucosylation patterns indicative of malignant potential.

Imaging of the ovary and adnexa: clinical issues and applications of MR imaging.

Many if not most adnexal masses that are resected for suspicion of malignancy are benign, and the ratio of benign to malignant masses removed increases dramatically if asymptomatic women are screened. More specific diagnostic tests may reduce the rate of unnecessary surgery in these patients. Magnetic resonance (MR) imaging may play a role in evaluating women with problematic ultrasound diagnoses. Conventional and chemical shift MR imaging can allow specific diagnosis of dermoid cysts, pedunculated leiomyomas, ovarian fibromas, most endometriomas, and hydrosalpinx. Gadolinium-enhanced MR imaging allows identification of papillary projections and septa in ovarian neoplasms and differentiation of clot and debris from vascularized tissue. Results of available studies suggest that MR imaging of the female pelvis is cost-effective in selected patients and can be used to obviate surgery in some patients. MR imaging with the use of these techniques may allow more appropriate clinical decisions to be made in selected patients with complex adnexal disease.

Fluorescence in situ hybridization analysis of trisomy 12 in ovarian tumors.

Conventional cytogenetic studies have suggested that trisomy 12 may be a characteristic nonrandom numerical chromosome anomaly in benign ovarian tumors, particularly sex cord-stromal tumors. To confirm this finding, and to avoid possible culture artifact introduced during cytogenetic analysis, the authors performed fluorescence in situ hybridization (FISH) in paraffin-embedded samples of select ovarian neoplasms. Forty-four ovarian fibromas and granulosa cell tumors and 31 benign and borderline epithelial ovarian tumors were examined for the presence of trisomy 12. Trisomy 12 was detected in 40% (8 of 20) of the fibromas. No evidence of trisomy 12 was present in 24 granulosa cell tumors, although 1 granulosa cell tumor was tetrasomic for chromosome 12. Trisomy 12 was found in 27% (3 of 11) of the serous borderline tumors, but was not observed in any of the benign epithelial tumors (13 serous and 7 mucinous cystadenomas). These results confirm that trisomy 12 occurs in a significant proportion of fibromas. However, the incidence of trisomy 12 in granulosa cell tumors is far lower than suggested by previous studies. These results, in conjunction with those of previous cytogenetic reports, suggest that trisomy 12 is rare in benign epithelial ovarian tumors, but occurs fairly commonly as a sole anomaly in borderline epithelial tumors. Further investigation is necessary to establish the significance of trisomy 12 in the pathogenesis of these tumors.

Nevoid basal cell carcinoma syndrome.

The nevoid basal cell carcinoma syndrome is an autosomal dominant disorder that predisposes to basal cell carcinomas of the skin, ovarian fibromas, and medulloblastomas. Unlike other hereditary disorders associated with cancer, it features widespread developmental defects. Laboratory studies of radiation sensitivity and chromosome instability over the past 20 years have generally yielded negative or inconclusive results. Recently, screening for allelic loss in sporadic and hereditary basal cell carcinomas, hereditary ovarian fibromas, and sporadic medulloblastomas provided evidence for a tumor suppressor gene on chromosome 9q, important in all three tumor types. Demonstration of a chromosome 9q deletion in an unusual patient with this syndrome and genetic linkage studies in large kindreds indicated that the nevoid basal cell carcinoma syndrome gene maps to the exact same location lost in tumors. These data show that tumors arise with homozygous inactivation of the gene and imply that it normally functions as a tumor suppressor. In contrast, hemizygous germ-line mutations lead to multiple congenital anomalies.

Nevoid Basal Cell Carcinoma Syndrome

The nevoid basal cell carcinoma syndrome is an autosomal dominant disorder that predisposes to basal cell carcinomas of the skin, ovarian fibromas, and medulloblastomas. Unlike other hereditary disorders associated with cancer, it features widespread developmental defects. Laboratory studies of radiation sensitivity and chromosome instability over the past 20 years have generally yielded negative or inconclusive results. Recently, screening for allelic loss in sporadic and hereditary basal cell carcinomas, hereditary ovarian fibromas, and sporadic medulloblastomas provided evidence for a tumor suppressor gene on chromosome 9q, important in all three tumor types. Demonstration of a chromosome 9q deletion in an unusual patient with this syndrome and genetic linkage studies in large kindreds indicated that the nevoid basal cell carcinoma syndrome gene maps to the exact same location lost in tumors. These data show that tumors arise with homozygous inactivation of the gene and imply that it normally functions as a tumor suppressor. In contrast, hemizygous germ-line mutations lead to multiple congenital anomalies.