Diagnosis, laparoscopic management, and histopathologic findings of juvenile cystic adenomyoma: a review of nine cases

doi:10.1016/j.fertnstert.2009.05.010

Fertility and Sterility

Volume 94, Issue 3, August 2010, Pages 862-868

https://doi.org/10.1016/j.fertnstert.2009.05.010 Get rights and content

Objective

To define diagnostic criteria for juvenile cystic adenomyoma (JCA), describe the histologic features of the condition and evaluate laparoscopic excision for treating associated dysmenorrhea and pelvic pain.

Design

Prospective long-term follow-up study.

Setting

University-affiliated hospital.

Patient(s)

Nine consecutive patients with JCA.

Intervention(s)

Patients meeting the diagnostic criteria for JCA underwent laparoscopic enucleation of the lesion. The severity of dysmenorrhea was evaluated before surgery and every 6 months after surgery. Five patients underwent second-look laparoscopy (SLL) 6 months after surgery.

Main Outcome Measure(s)

Relief of dysmenorrhea as measured by a visual analog scale, postoperative healing at SLL, and subsequent pregnancy when desired by the patient.

Result(s)

Laparoscopic enucleation of the cystic adenomyoma resulted in a statistically and clinically significant reduction in dysmenorrhea and improved chronic pelvic pain. Neither cystic adenomyoma nor severe dysmenorrhea recurred during the follow-up period. Adhesions were minimal at SLL. Two of the three patients who desired pregnancy conceived after surgery. The histologic findings of the JCA lesion and adenomyosis were similar, and the endometrial glands and stroma infiltrating the surrounding myometrium in all patients were consistent with the appearance of adenomyosis.

Conclusion(s)

We defined the diagnostic criteria for JCA, and demonstrated significant improvement of dysmenorrhea after laparoscopic excision of the lesion.

Section snippets

Materials and methods

We enrolled nine consecutive patients with cystic adenomyoma, diagnosed with JCA according to our proposed criteria, at our hospital between 2000 and 2007. Each of them provided written informed consent to undergo laparoscopic tumor enucleation at our department (Table 1), and the Ethics Committee of Juntendo University approved the study protocol. Cystic adenomyoma was diagnosed by transvaginal ultrasonography (TVUS) and MRI (Fig. 1). Associated urogenital anomalies were checked using

Symptoms (Table 1)

The age at onset of dysmenorrhea in the patients with JCA was 19.3 ± 4.2 years (mean ± SD). In general, JCA develops within 15 years of menarche. The condition was diagnosed in our patients at a mean ± SD of 6.6 ± 4.3 years after menarche, and most of them described the severity of dysmenorrhea as being 8–10 on the VAS. Reports indicate that symptoms other than dysmenorrhea can develop over time, such as hypogastric pain and lumbago during menstruation. Indeed, all of our patients had these

Diagnostic Criteria for JCA

The mean ± SD age of our nine patients was 25.2 ± 4.4 years (range 20 to 30 years). Although most patients described in earlier studies were ≤20 years of age, our experience indicated that the diagnostic criteria for JCA should include women ranging from adolescence to 30 years of age. Furthermore, the cystic lesions of JCA have similar characteristics on TVUS and MRI in appearance and location regardless of age. The interval from menarche to the appearance of JCA symptoms tended to be longer

Conclusion

Juvenile cystic adenomyoma is defined as a solitary myometrial cyst measuring ≥1 cm that is surrounded by hypertrophic endometrium, independent of the uterine lumen, and presents in women ≤30 years of age in association with severe dysmenorrhea. Laparoscopic excision can significantly improve the associated dysmenorrhea and increase the likelihood of successful pregnancy. The condition might represent a larger more discrete variant of adenomyosis among young women. Similar disease entities have

Acknowledgments

The authors thank Dr. Toshio Matsumoto for advice regarding pathologic terminology.

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Cystic adenomyosis is a rare benign lesion in women of reproductive age that is known to cause hypermenorrhea, dysmenorrhea, and abnormal uterine bleeding. Our case represents the second documented case of diverticulum-like adenomyosis. However, the patient in our case did not exhibit abnormal uterine bleeding or dysmenorrhea. One possible explanation for this finding is that the sinus tract was too small to cause blood influx into the uterine cavity.
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We present a 16-year-old patient with chronic pelvic pain and ultrasound evidence of a 2.4 cm adenomyoma. The lesion was thought specifically to represent a juvenile cystic adenomyoma, defined as a cystic lesion >1 cm occurring in women younger than 30 years with severe dysmenorrhea that is distinct from the uterine cavity and surrounded by hypertrophic myometrium.
Given minimal relief from medical therapy and high suspicion for coexistent endometriosis, our patient elected to undergo laparoscopic resection of adenomyoma and excision of pelvic lesions.
Preoperative considerations discussed in this video include imaging to identify the location of the lesion and adjacent structures, such as the uterine vessels, discontinuation of gonadotropin-releasing hormone agonist for adequate intraoperative visualization, and the high likelihood of encountering endometriosis at operation.
We review the following surgical techniques: maximize visualization with the use of a uterine manipulator and temporary oophoropexy, optimize hemostasis via temporary uterine artery ligation and control of collateral blood vessels, complete ureterolysis, meticulous enucleation of adenomyoma, and excision of coexistent endometriotic lesions. Surgical findings demonstrated a 2 cm lesion along the left lower uterine segment and red-brown lesions along bilateral ovarian fossa, pathologically confirmed as adenomyoma and superficial endometriosis, respectively.
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Técnicas quirúrgicas para la escisión de adenomioma quístico juvenil.
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Presentamos una paciente de 16 años con dolor pélvico crónico y evidencia ecográfica de un adenomioma de 2,4 cm. La lesión se pensó que representaba específicamente un adenomioma quístico juvenil, definido como una lesión quística >1 cm que ocurre en mujeres menores de 30 años con dismenorrea severa que es distinta de la cavidad uterina y rodeada por miometrio hipertrófico.
Dado el alivio mínimo de la terapia médica y la alta sospecha de endometriosis coexistente, nuestra paciente eligió someterse a una resección laparoscópica del adenomioma y escisión de las lesiones pélvicas.
las consideraciones preoperatorias discutidas en este video incluyen imágenes para identificar la ubicación de la lesión y estructuras adyacentes, como los vasos uterinos, interrupción del agonista de la hormona liberadora de gonadotropina para una adecuada visualización, y la alta probabilidad de encontrar endometriosis en la operación.
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Citation Excerpt :
On the contrary, large cysts, termed as an adenomyotic cyst, cystic adenomyosis, juvenile cystic adenomyosis/adenomyoma (JCA), or adenomyoma, can be found within the uterine myometrium, with adjacent smooth muscle hypertrophy, of adolescents and women <30 years. Although the first published report on JCA comes from Tamura et al., in 1996 [4], Takeuchi et al., in 2009 defined the diagnostic criteria of JCA based on age (<30 years), presence of cystic lesion ≥1 cm in diameter independent of the uterine lumen and covered by hypertrophic myometrium on diagnostic images, and associated with severe dysmenorrhea [5]. Although some women may be asymptomatic, most women present with severe dysmenorrhea, pelvic pain, menorrhagia, uterine enlargement, and infertility [6].
Juvenile cystic adenomyoma (JCA) is a rare uterine pathology with <40 cases reported in the current literature since 1996 when Tamura described it. We report a 13-year-old girl with a history of chronic pelvic pain and dysmenorrhea for 12 months. After diagnostic evaluation and identification of the cystic structure (∼6 cm) within the myometrium, fertility-sparing surgery was successfully performed. Histopathological examination of the uterine cyst was consistent with the diagnosis of JCA. The postoperative course was uneventful, and the patient was disease-free three years after surgery. JCA is a rare condition but should be considered in the differential diagnosis in adolescents with moderate to severe dysmenorrhea. Despite diagnostic advances, the awareness of the disorder remains low. Thus, our case report aims to increase awareness of this rare pathology with unclear etiology.
A rare case of the new entity of müllerian anomalies mimicking the noncommunicating rudimentary cavity with hemi-uterus: accessory cavitated uterine mass
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A 17-year-old virgin girl with chronic pelvic pain and severe progressive dysmenorrhea despite using nonsteroidal anti-inflammatory drugs and oral contraceptives since her menarche. We could not perform transvaginal examination and ultrasonography because of the virginity of the girl. Transabdominal ultrasound revealed a 24 × 24-mm heterogeneous cystic mass on the left adnexal region suggesting a noncommunicating functional rudimentary horn. This mass was within the left lateral uterine myometrial wall, and otherwise, the uterus and endometrial cavity seemed normal on ultrasonographic examination. Magnetic resonance imaging showed a cavitated nodule of 25 mm with hemorrhagic components inside the mass on the left side of the uterus and the normal endometrial cavity with a normal size uterus. After detailed evaluation and counseling, a decision was taken to perform laparoscopy.
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On laparoscopic exploration, we first observed a globular enlargement on the left wall of the uterus under the round ligament. Both the ovaries and the uterus were normal, and there were two normally developed fallopian tubes with no abnormality communicating with the normal uterus. The mass had irregular muscle fibers and ambiguous limits for detaching from the normal myometrial tissue. Afterward, the mass was resected completely without entering the endometrial cavity. It was thought that these irregular borders may be a factor of confusion as a degenerated myoma on the diagnosis. The blunt and sharp dissection methods were used bidirectionally, and finally, the lesion was enucleated. The mass was incised, and chocolate-like brownish fluid spilled out from the cavity of the mass. The endometrium-like epithelium and surrounding muscular layer were seen on the mass. The myometrial defect was approximated and closed with a polyglactin absorbable suture. Histopathological examination revealed the endometrial lining cavitated mass surrounded by myometrium, and thus, it was consistent with the diagnosis of ACUM.
The postoperative period of the patient was uneventful. She was discharged without any complaint. Her severe pelvic pain and symptoms of dysmenorrhea were resolved 1 month after the surgery. We did not prescribe any medication for menstrual suppression and pain management before or after surgery. During the follow-up period, she had a regular menstrual cycle and no pelvic pain and dysmenorrhea for 2 years.
Accessory cavitated uterine mass is a new entity having a normal endometrial cavity and is different from other müllerian duct anomalies. Severe dysmenorrhea and chronic pelvic pain are early-onset, and they are persistent despite using medications, but suspecting this problem is the main determinant for confirming the diagnosis. The final diagnosis of ACUM can be made after resecting the mass and confirming with histopathological examination. Accessory cavitated uterine mass may be confused with a similar lesion of juvenile cystic adenomyosis (JCA). They present very similar clinical and pathological findings, but JCA is a rare form of focal adenomyosis that contains cystic contents inside the lesion, and it has more intense adenomyosis surrounding the endometrial lining of a cavitated mass. In our case, histopathological examination revealed muscle fibers around the endometrial epithelium, with no adenomyotic foci. It was so difficult to distinguish these two similar lesions during the surgery. Thus, we could distinguish ACUM from JCA with this pathological confirmation. Accessory cavitated uterine mass may also be misdiagnosed as U4a of ESHRE/ESGE classification and 2b of ASRM classification, but it should be distinguished from these anomalies. Moreover, it is important to define ACUM clearly in the U4 or U6 class of the ESHRE/ESGE and 2b of ASRM classification systems.
Un caso raro de una nueva entidad de malformación mülleriana que imita un hemiútero con cavidad rudimentaria no comunicante: masa uterina cavitada accesoria.
Demostrar la escisión laparoscópica de una masa uterina cavitada accesoria (ACUM) y mostrar que las anomalías atípicas pueden conducir a un diagnóstico erróneo de un cuerno rudimentario no comunicante
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Hospital universitario
Niña virgen de 17 años de edad con dolor pelviano crónico y dismenorrea severa progresiva a pesar del uso de drogas antinflamatorias no esteroideas y anticonceptivos orales desde su menarca. No pudimos efectuar examen ni ecografía transvaginal por la virginidad de la niña. La ecografía transabdominal reveló una masa quística heterogénea de 24 x 24 mm sobre la región anexial izquierda sugestiva de cuerno rudimentario funcional no comunicante. Esta masa estaba adentro de la pared miometrial uterina lateral izquierda, y por lo demás, el útero y la cavidad endometrial se veían normales en el examen ecográfico. Las imágenes de la resonancia magnética mostraron un nódulo cavitado de 25 mm con componentes hemorrágicos en el interior de la masa del lado izquierdo del útero y la cavidad endometrial normal con un útero de tamaño normal. Luego de una evaluación detallada y asesoramiento, se tomó la decisión de realizar una laparoscopía.
Resección laparoscópica de una ACUM
En la exploración laparoscópica, observamos primero un agrandamiento globular en la pared izquierda del útero bajo el ligamento redondo. Tanto los ovarios como el útero fueron normales, y había dos trompas de Falopio desarrolladas normalmente sin anomalías que se comunicaban con el útero normal. La masa tenía fibras musculares irregulares y límites ambiguos para separarse del tejido miometrial normal. Posteriormente, la masa se resecó completamente sin entrar en la cavidad endometrial. Se pensó que estos bordes irregulares pueden ser un factor de confusión, como un mioma degenerado en el diagnóstico. Se utilizaron métodos de disección roma y cortante bidireccionalmente y finalmente se enucleó la lesión. Se incidió la masa y se derramó un líquido parduzco parecido al chocolate de la cavidad de la masa. Se observaron en la masa epitelio similar al endometrio y capa muscular circundante. El defecto en el miometrio fue aproximado y cerrado con una sutura absorbible de poliglactina. El examen histopatológico reveló el revestimiento endometrial de la masa cavitada de miometrio y, por lo tanto, era consistente con el diagnóstico de ACUM.
El período postoperatorio de la paciente fue sin incidentes. Fue dada de alta sin ninguna queja. Su dolor pelvico severo y los síntomas de dismenorrea se resolvieron al mes luego de la cirugía. No le prescribimos ninguna medicación para suprimir la menstruación ni para el manejo del dolor antes o después de la cirugía. Durante el período de seguimiento, tuvo ciclos menstruales regulares sin dolor pelviano ni dismenorrea por 2 años.
La masa uterina cavitada accesoria es una nueva entidad que tiene una cavidad endometrial normal y es distinta de otras malformaciones de los conductos müllerianos. La dismenorrea severa y el dolor pelviano crónico son de aparición temprana y persistentes a pesar del uso de medicamentos, pero la sospecha de este problema es el mayor determinante para confirmar el diagnóstico. El diagnóstico final de ACUM puede hacerse luego de resecar la masa y la confirmación con el examen anatomopatológico. La masa uterina cavitada accesoria puede ser confundida con una lesión similar de adenomiosis quística juvenil (JCA). Ésta, presenta hallazgos clínicos y patológicos muy similares, pero la JCA es una forma rara de adenomiosis focal que contiene quistes en su interior, y tiene intensa adenomiosis que rodea el revestimiento endometrial de la masa cavitaria. En nuestro caso, el examen histopatológico reveló fibras musculares alrededor del epitelio endometrial, sin focos de adenomiosis. Fue muy difícil distinguir entre estas dos lesiones similares durante la cirugía. Por lo tanto, podríamos distinguir ACUM de JCA con la confirmación patológica. La masa uterina cavitada accesoria puede también diagnosticarse erróneamente como U4a de la clasificación ESHRE/ESGE y 2b de la clasificación de ASRM. Pero debe ser distinguida de esas anomalías. Además, es importante definir claramente ACUM en U4 o U6 de los sistemas de clasificación de ESHRE/ESGE y 2b del de ASRM.

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: H.T. has nothing to disclose. M.K. has nothing to disclose. I.K. has nothing to disclose. J.K. has nothing to disclose. K.K. has nothing to disclose. M.J. has nothing to disclose.

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EndometriosisDiagnosis, laparoscopic management, and histopathologic findings of juvenile cystic adenomyoma: a review of nine cases

Objective

Design

Setting

Patient(s)

Intervention(s)

Main Outcome Measure(s)

Result(s)

Conclusion(s)

Section snippets

Materials and methods

Symptoms (Table 1)

Diagnostic Criteria for JCA

Conclusion

Acknowledgments

Best Pract Res Clin Obstet Gynaecol

J Am Assoc Gynecol Laparosc

J Minim Invasive Gynecol

Fertil Steril

J Minim Invasive Gynecol

Fertil Steril

Fertil Steril

Am J Obst Gynec

Endometriosis
Diagnosis, laparoscopic management, and histopathologic findings of juvenile cystic adenomyoma: a review of nine cases