David Cohen, M.D.·Published 19 March 2008·10 min read
Key points
Transvaginal ultrasonography, needle biopsy, and MRI often can't distinguish adenomyosis from fibroids.
Traditionally a clinician will make the diagnosis retrospectively or it's an incidental finding by the pathologist at the time of hysterectomy.
Eighty percent of adenomyosis cases are reported in women between 40 and 50 years old.
Adenomyosis at hysterectomy: a study on frequency distribution and patient characteristics.
Transvaginal ultrasonography, needle biopsy, and MRI often can't distinguish adenomyosis from fibroids. Classical signs like uterine enlargment, pelvic pain, and abnormal menstrual bleeding are not pathognomic.
With no clear-cut set of signs and symptoms to advertise its presence, experts suggest diagnosis may require MRI, transvaginal U/S, and/or needle biopsy. Among your treatment options: hysterectomy, GnRH agonists, and the levonorgestrel IUD.
A denomyosis, first described in 1860, is a common nonmalignant condition of the uterus characterized by the presence of endometrial glands and stroma ectopically located in the myometrium. How common is it?
The disease has been reported in 54% of uteri at necropsy. And because most studies have attempted to calculate its incidence from unsolicited hysterectomized specimens, we don't really have an accurate estimate of how common the disease is in the general population. The reported incidence also varies widely depending on what definition is used to define the disease. The incidence is directly proportional to the number of histologic sections that are examined, and depends on how carefully the pathologist searches for it. One investigator, for instance, found that it occurred in 31% of hysterectomized specimens where three routine sections were performed, but increased to 61% with six sections. So it's not surprising that the incidence of adenomyosis reported in the literature ranges from 8.8% to 61.5%.
Similarly, the exact pathogenesis of adenomyosis remains a mystery. Some researchers theorize that a disruption occurs in the basalis layer of the endometrium, allowing the glands and stroma to invade the myometrium. This causes hyperplasia and hypertrophy of the myometrium, enlarging the uterus. But how this disruption occurs remains unclear. Some suspect that surgical trauma triggers it. Prior uterine surgery—in particular dilation and curettage associated with spontaneous and induced abortion—increases the risk of adenomyosis. But this alone cannot explain adenomyosis because the disease is known to exist in women who have never undergone uterine surgery. High estrogen levels have also been implicated as the mechanism of myometrial invasion. We know, for instance, that parity has been linked to adenomyosis, and it may be directly related to the number of live births. Endometrial hyperplasia, an estrogen-dependent lesion, has also been linked to adenomyosis in some studies.
How do you make the diagnosis?
Traditionally a clinician will make the diagnosis retrospectively or it's an incidental finding by the pathologist at the time of hysterectomy.
The standard diagnostic criterion is the finding of endometrial glands and stroma located more than one low-powered field (2.5 mm) from the basalis layer of the endometrium. However, this definition varies widely, with nine definitions seen in our literature search.
Written and revised by
David Cohen, M.D.
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Although adenomyosis may cause uterine enlargement, the organ does not become massive. It usually weighs between 80 and 200 g, unless fibroids are also present. Upon gross evaluation the myometrial wall appears thickened and often contains small chocolate-colored areas, which represent islands of endometrial bleeding.
TWO VARIETIES OF ADENOMYOSIS have been described. The most common type, occurring in two thirds of cases, is a diffuse disease resulting in generalized enlargement of both anterior and posterior walls of the uterus. The second presentation, occurring in one third of the cases, is a focal disease. Often there are distinct unencapsulated areas of adenomyosis within the myometrium referred to as adenomyomas.
Although the disease is difficult to diagnose in vivo, a few radiographic techniques have been making some headway. Since patients suspected of having adenomyosis preoperatively might have other pathologies contributing to their symptoms, it is important to differentiate them from one another because treatment options can vary. Some co-existing conditions, such as dysfunctional uterine bleeding, may not require very aggressive treatment.
• A number of studies have proposed transvaginal ultrasonography (TVU), needle biopsies, and magnetic resonance imaging (MRI) to detect adenomyosis.
The problem with these measures is that they are far from perfect; sensitivities and specificities vary widely from study to study. And these diagnostic tools often cannot distinguish fibroids from adenomyosis, one of the main reasons for trying to establish a pretreatment diagnosis.
• Attempts have been made to diagnose the condition preoperatively by transcervical needle biopsy of the myometrium.
However, even with multiple needle biopsies, the test's sensitivity (45%) is too low to be of practical clinical value. Other researchers have suggested that TVU may be used to diagnose adenomyosis. They suggest U/S may reveal irregular cystic spaces, heterogenic lesions, myometrial echoes with indistinct margins, mottled texture, and linear striations. A wide range of sensitivities have been reported (53%–70%). underscoring the limited value of this approach. Another disadvantage of TVU is that its accuracy in making the diagnosis depends on the operator and patient.
• An increasing number of clinicians are using MRI to detect adenomyosis and it appears to be the best way to make the diagnosis. {pagebreak} One research team prospectively compared TVU to MRI in diagnosing adenomyosis preoperatively. MRI was significantly superior (P< 0.02) Another investigator reported MRI sensitivities ranging from 70% to 82%, specificities 84% to 92%, with the positive predictive value of 58% to 83% and a negative predictive value of 86% to 95%. The approach has also been validated in a study to differentiate adenomyosis from fibroids. The typical appearance on a T2 weighted MRI is a widened band of low intensity in the junctional zone (JZ) of the uterus. A width of greater than 5 to 20 mm is considered significant, but this is also debated. Advantages of MRI include that it is not operator dependent, the images are reproducible, and it is not associated with ionizing radiation; disadvantages are the high cost, limited availability, the lack of consensus of how wide the JZ should be, and low sensitivity.
The literature has also suggested that adenomyosis, often referred to as endometriosis interna, is associated with, if not a variant of, endometriosis, and some have suggested that the two entities usually occur concomitantly. The evidence, however, doesn't support this notion. Prior controlled studies reported prevalence of endometriosis between 5% to 10% in specimens with adenomyosis and 7% to 8% without adenomyosis. These differences were not statistically significant. The common feature in both diseases is the presence of ectopic endometrial glands and stroma, but this similarity does not necessarily indicate a common cause. These findings suggest that adenomyosis is not more common in the presence of endometriosis and that they are most likely clinically different diseases.
ADENOMYOSIS IS OFTEN found in hysterectomy specimens of women with concurrent fibroids. The reported incidence of concurrent fibroids has ranged from 19% to 57%. Many authors have concluded that this high prevalence reflects an association between adenomyosis and fibroids. However, the majority of these studies did not examine the incidence of fibroids in control specimens, that is, in women without adenomyosis. Two previous authors, Vercellini et al. and Shaikh et al., concluded that fibroids are equally common in specimens with and without adenomyosis.
What symptoms should you expect?
Eighty percent of adenomyosis cases are reported in women between 40 and 50 years old. There is no consistent constellation of symptoms that clearly identify adenomyosis.
In fact, most women are asymptomatic. Classically it has been described to present as a triad: uterine enlargement, pelvic pain, and heavy, abnormal menstrual bleeding. As you might expect, the severity of symptoms correlates with the extent of disease present. When present, these symptoms lead to a presumptive preoperative diagnosis and are often used as an indication for hysterectomy. But keep in mind that 80% of patients with adenomyosis also have another pathologic process present in the uterus that often obscures the diagnosis of adenomyosis.
None of these symptoms, or the triad alone, is pathognomonic for adenomyosis, and attributing the symptoms solely to adenomyosis, and not the other pathologies, may be a mistake. Many symptoms may be due to other concurrent conditions such as fibroids, or dysfunctional uterine bleeding. {pagebreak} What's the best treatment approach?
The definitive treatment for adenomyosis is hysterectomy.
Unlike fibroids, where a myomectomy is a surgical option, adenomyosis cannot be easily excised. Until a screening test exists that has acceptable sensitivity and specificity, safety profile, and cost, it appears that adenomyosis can only be accurately diagnosed and treated if a hysterectomy is simultaneously performed. Given that patients with symptoms suggestive of adenomyosis probably have other pathologies contributing to their symptoms, we suggest that treatment be targeted to the patient's symptoms or other conditions. The physician needs to consider each patient's individual situation, and make decisions based on what is appropriate for the woman's age, parity, and plans for future reproduction.
IF A PATIENT IS NOT a candidate for hysterectomy, or doesn't want one, you can attempt medical management of the symptoms. Adenomyosis is known to have both estrogen and progesterone receptors present, which is why hormone therapy has been investigated as an option. Since researchers have proposed that estrogen may be involved in the disease's pathophysiology, suppressing the estrogenic environment improves the symptoms.
Investigators have also reported that GnRH-agonists can relieve adenomyosis. They have been shown to reduce uterine size, alleviate pelvic symptoms, and decrease the JZ on MRI. This may be a good option for women who temporarily want to delay surgery or who desire children in the future. Pregnancies have been reported after treatment cessation, in histologically confirmed cases of adeno-myosis, after treatment with a GnRH-agonist. It is likely that when depot preparations of GnRH antagonists become available that they would be more efficacious than GnRH agonists since they will not cause an initial gonadotropin flare. The downside, however, is that antagonists should not be used for more than 6 months because the subsequent hypoestrogenism can compromise bone density. The levonorgestrel-releasing intrauterine device has also been used successfully in women with adenomyosis. It has been observed to decrease uterine size, improve anemia, and relieve menorrhagia. Advantages of this is that it can be used on a long-term basis, and fertility is retained.
While adenomyosis may in fact be more common that we previously thought, its presence may not be clinically significant since it is often asymptomatic and frequently concurrent with other symptom producing conditions. As more controlled studies are done, we may be able to determine the significance of the diagnosis, if any.
DR. MASEELALL is a Fellow, Reproductive Endocrinology and Infertility, at UMDNJ-New Jersey Medical School, Newark, NJ. DR. WEISS is Professor and Chair, Department of Obstetrics, Gynecology and Women's Health, UMDNJ-New Jersey Medical School, Newark, NJ.
REFERENCES
1. Lewinski H. Beitrag zur frage der adenomyosis. Zentralbl Gynaekol. 1931;55:2163-2167.
2. Bird CC, McElin TW, Manalo-Estrella P. The elusive adenomyosis of the uterus—revisited. Am J Obstet Gynecol. 1972;112:583-593.
3. Vercellini P, Parazzini F, Oldani S, et al. Adenomyosis at hysterectomy: a study on frequency distribution and patient characteristics. Hum Reprod. 1995;10:1160-1162.
4. Shaikh H, Khan KS. Adenomyosis in Pakistani women: four year experience at the Aga Khan University Medical Centre, Karachi. J Clin Pathol. 1990;43:817-819.
5. Molitor JJ. Adenomyosis: a clinical and pathological appraisal. Am J Obstet Gynecol. 1971;110:275-284.
6. Kilkku P, Erkkola R, Gronroos M. Non-specificity of symptoms related to adenomyosis. A prospective comparative survey. Acta Obstet Gynecol Scand. 1984;63:229-231.
7. Curtis KM, Hillis HD, Marchbanks PA, et al. Endometrial-myometrial border during pregnancy as a risk factor for adenomyosis. Am J Obstet Gynecol. 2002;187:543-544.
8. Levgur M, Abadi MA, Tucker A. Adenomyosis: symptoms, histology, and pregnancy terminations. Obstet Gynecol. 2000;95:688-691.
9. McElin TW, Bird CC. Adenomyosis of the uterus. Obstet Gynecol Annu. 1974;3:425-441.
10. Emge LA. The elusive adenomyosis of the uterus. Its historical past and its present state of recognition. Am J Obstet Gynecol. 1962;83:1541-1563.
11. Mathur BB, Shah BS, Bhende YM. Adenomyosis uteri. A pathologic study of 290 cases. Am J Obstet Gynecol. 1962;84:1820-1829.
12. Vercellini P, Cortesi I, De Giorgi O, et al. Transvaginal ultrasonography versus uterine needle biopsy in the diagnosis of diffuse adenomyosis. Hum Reprod. 1998;13:2884-2887.
13. Lone FW, Balogun M, Khan KS. Adenomyosis: not such an elusive diagnosis any longer. J Obstet Gynaecol. 2006;26:225-228.
14. Ascher SM, Arnold LL, Patt RH, et al. Adenomyosis: prospective comparison of MR imaging and transvaginal sonography. Radiology. 1994;190:803-806.
15. Togashi K, Ozasa H, Konishi I, et al. Enlarged uterus: differentiation between adenomyosis and leiomyoma with MR imaging. Radiology. 1989;171:531-534.
16. Benson RC, Sneeden VD. Adenomyosis: a reappraisal of symptomatology. Am J Obstet Gynecol. 1958;76:1044-1057.
17. Grow DR, Filer RB. Treatment of adenomyosis with long-term GnRH analogues: a case report. Obstet Gynecol. 1991;78:538-539.
18. Huang FJ, Kung FT, Chang SY, et al. Effects of short-course buserelin therapy on adenomyosis. A report of two cases. J Reprod Med. 1999;44:741-744.
19. Imaoka I, Ascher SM, Sugimura K, et al. MR imaging of diffuse adenomyosis changes after GnRH analog therapy. J Magn Reson Imaging. 2002;15:285-290.
20. Fedele L, Bianchi S, Raffaelli R, et al. Treatment of adenomyosis-associated menorrhagia with a levonorgestrel-releasing intrauterine device. Fertil Steril. 1997;68:426-429.
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