Prenatal diagnosis of an adrenal mature teratoma mimicking a neuroblastoma

doi:10.21203/rs.3.rs-1828520/v1

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Case Report

Prenatal diagnosis of an adrenal mature teratoma mimicking a neuroblastoma

https://doi.org/10.21203/rs.3.rs-1828520/v1

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Aim of the study: To present our experience with an adrenal antenatal mass firstly diagnosed as a left adrenal neuroblastoma that turned out to be a mature teratoma after anatomopathology.

Case description: We present the case of an infant with an antenatal diagnosis of a left adrenal cystic image on the 22nd week of amenorrhea ultrasound. The fetal MRI showed a 20 x 13 mm non-calcified cystic formation of the left adrenal gland, compatible with a neuroblastoma.

At birth, the diagnosis has been confirmed by an ultrasound that showed an anechogenic image of the left adrenal gland of 17 x 12 mm, with thin walls.

Besides, in the 9 months interval between birth and the last CT scan, the image had minimally diminished in size. Then it stabilized at about 2 cm.

The workup was otherwise normal.

The infant was closely monitored during his first year and in the absence of significant regression of the adrenal mass, we decided to perform a left laparoscopical adrenalectomy.

Unexpectedly, the anatomopathologist results showed a mature cystic adrenal teratoma.

Conclusions: An adrenal mass diagnosed antenatally is generally a haemorrhage or a neuroblastoma. Only two other case of unexpected adrenal teratoma in infants are described in literature. Adrenal teratomas are very rare and those diagnosed antenatally even more. At present, we have no clinical, biological, or radiological evidence to suspect them before surgical removal.

adrenal mass

mature teratoma

antenatal diagnosis

We report the case of an antenatally detected left adrenal cystic mass that was initially managed like congenital neuroblastoma, which turned out to be a mature teratoma post-adrenalectomy.

Antenatally detected suprarenal masses are likely to be neuroblastoma or adrenal haemorrhage, but may be rare benign lesions (subdiaphragmatic extralobar pulmonary sequestration, bronchogenic cyst, renal dysplasia) [Oguzkurt P2009].

Teratomas typically involve the gonads or the sacrococcygeal region and are rarely retroperitoneal.

Prenatally detected adrenal teratomas are extremely uncommon, we therefore decided to describe our recent experience and to review the literature.

A male infant with a history of a prenatally detected left suprarenal mass was addressed to the surgical unit. Antenatal ultrasonography at 22 weeks of gestation disclosed a cystic left suprarenal mass about 2 cm in diameter. The fetal MRI showed a 20 x13 mm non-calcified cystic formation of the left adrenal gland, compatible with a neuroblastoma. FIG 1

Vaginal delivery was uneventful.

Results of routine laboratory tests including complete blood count, blood biochemistry, and urinalysis were normal, with the exception of a major ferroprive anaemia.

Ultrasonography at 1 months of life showed an anechogenic image of the left adrenal gland of 17 x 12 mm, with thin walls. FIG 2A

In the 9 months interval between the birth’s ultrasound and the last imaging, the mass minimally diminished in size and it stabilized at about 2 cm.

The infant was closely monitored during his first year of life and in the absence of symptomatology or significant regression of the mass, we decide to proceed with its removal.

We perform a preoperative CT showed a partially cystic 20 x 22 mm mass in the left adrenal region, non-enhanced, without calcification. FIG 2B

It was therefore decided to remove surgically the tumor. An uneventful adrenalectomy was performed by laparoscopy.

The patient was monitored for 24 hours, restarted alimentation immediately after surgery, and was discharged in good condition at 48 hours.

The patient had no symptoms during 5 months of follow-up.

The anatomopathologist result showed a mixed composition of ciliated columnar epithelium of the respiratory type, well differentiated cartilaginous structures as well as small seromucous glands. The specimen was diagnosed as a mature cystic adrenal teratoma, of complete exeresis and without any suspicious sign of malignancy. FIG 3

Adrenal masses could be diagnosed from 20 weeks of gestational age when adrenal glands turn visible sonographically. It most often consists of haemorrhage or neuroblastoma [Yao W 2013] but other differential diagnoses are represented by adrenal cysts, subdiaphragmatic extralobar pulmonary sequestrations, bronchogenic cyst, hepatic tumors, adrenogenital syndrome (secondary to congenital adrenal hyperplasia) or renal dysplasia. [Suk-Bae Moon]

It still remains a challenge for clinicians to distinguish the nature of asymptomatic adrenal mass based only on antenatal imaging findings. The accurate diagnosis of neonatal adrenal mass depends mostly on their dynamic observation by imaging methodical like enhanced CT and ultrasonography.

Several studies suggest that prenatal diagnosis of suprarenal masses should be preferentially focused on neuroblastoma because usually they prove to be congenital neuroblastomas. [Yao W2012]

For this reason, we initially managed our patient like a congenital neuroblastoma. We based our workup only on ultrasound monitoring, accordingly with several authors. As a matter of fact, congenital neuroblastoma generally has a favourable prognosis with regression of lesions in most cases during the first year of life. [HeroB 2008] [Sperling J 2015]

We decided not to perform any specific blood test to support our diagnosis. In fact, catecholamine metabolites often demonstrated to be not a valuable index for the diagnosis of adrenal masses [Yao W2012]. Also the role of AFP is controversial: AFP levels are normally very high at birth and fall exponentially to normal adult levels by the age of 1 year.

In our case, was the atypical aspect at the imaging and above all the atypical behaviour (lack of regression, stability of lesion) that take us to perform an adrenalectomy.

We were rather surprised by the result of the anatomopathologist analysis that showed a mixed composition of ciliated columnar epithelium of the respiratory type, well differentiated cartilaginous structures and seromucous glands, diagnosed as a mature cystic adrenal teratoma.

Teratomas are rare germ cell neoplasms that originates from totipotent cells that differentiate into tissue components representing derivatives of ectoderm, mesoderm, and endoderm. Commonly they affect the gonads but also may occur at extragonadal sites. The common extragonadal sites include anterior mediastinum, sacrococcygeal region, pineal region and retroperitoneum.

The retroperitoneal localisation is extremely rare, above all in childhood.

To the best of our knowledge, only very few other cases of primary mature adrenal teratomas affecting children are reported in Literature. Most of these cases were incidentally detected or clinically manifested because of abdominal distension. Is extraordinarily rare that the tumor is diagnosed antenatally. To date only two other case of adrenal antenatal mass firstly diagnosed as neuroblastoma turned out to be a mature teratoma after anatomopathology are reported in Literature. [Oguzkurt P2009] [Yao W2013]

The most characteristic imaging feature of mature teratomas is a heterogeneous mass containing fluid, fat, and calcification. None of these features are considered diagnostic for teratomas: they are non-specific and it is rather the dynamic observation by CT and ultrasound that can help for differential diagnosis. [Haddad SE2020]

Mature teratoma’s prognosis is generally optimal, especially if the tumor is completely removed: complete surgical excision provides the best chance of cure. Therefore, the correct diagnosis is essential for optimal postnatal treatment, but it still remains a challenge.

It still remains a challenge for clinicians to distinguish the nature of asymptomatic adrenal mass. Surrenal teratomas are very rare and those of antenatal diagnosis even more. At present, we have no clinical, biological, or radiological evidence to suspect it before surgical removal, but they should be included in the differential diagnosis of a prenatally detected surrenal masses, and resection is advised.

Ethics approval and consent to participate : Informed consent was properly documented by both the patient’s parents. Ethical approval by the local hospital ethical committee was given properly.

Consent for publication : Yes, All authors read and approved the final manuscript.

Availability of data and materials : All data generated or analysed during this study are included in this article (ant its supplementary information files.)

Competing interests : The authors have no financial or personal relationships with people or organizations that could inappropriately influence or bias their work.

Funding : None

Authors' contributions : All authors substantially contributed to the manuscript. Olivier Abbo and Giulia Fusi performed surgical resection and perioperative care. Marion Gambart and Agnès Sartor decided and approved the treatments. Giulia Fusi and Camille Garcia were major contributors in writing the manuscript. All authors read and approved the final manuscript.

Acknowledgements : None

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Haddad SE, Hessissen L, Kababri ME, Lamalmi N, Kisra M, Allali N, Chat L. Primary mature adrenal teratoma in infant. Pan Afr Med J. 2020, Sep 7;37:27
Li Y, Zhong Z, Zhao X. Primary mature teratoma presenting as an adrenal tumor in a child. Urology. 2011 Sep;78(3):689-91
Yao W, Li K, Xiao X, Zheng S, Chen L. Neonatal suprarenal mass: differential diagnosis and treatment. J Cancer Res Clin Oncol. 2013 Feb;139(2):281-6.
Suk-Bae Moon, Hyun-Baik Shin, Jeong-Meen Seo, Suk-Koo Lee, Clinical features and surgical outcome of a suprarenal mass detected before birth
Vasies I, Brasseur-Daudruy M, Lempicki M, Vivier PH, Pirot N, Deurloo EE, Smets AMJB, Dacher JN. Imagerie des surrénales chez l'enfant.2015
Sperling J, Adrenal neuroblastoma, American Journal of Obstetrics and Gynecology, Volume 225, Issue 5, 2021, Pages B5-B6
Hero B, Simon T, Spitz R, Ernestus K, Gnekow AK, Scheel-Walter HG, Schwabe D, Schilling FH, Benz-Bohm G, Berthold F. Localized infant neuroblastomas often show spontaneous regression: results of the prospective trials NB95-S and NB97. J Clin Oncol. 2008 Mar 20;26(9):1504-10
Khong PL, Lam KY, Ooi CG, Liu MJ, Metreweli C. Mature teratomas of the adrenal gland: imaging features. Abdom Imaging. 2002 May-Jun;27(3):347-5

No competing interests reported.

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Prenatal diagnosis of an adrenal mature teratoma mimicking a neuroblastoma

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