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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1040

Sacrococcygeal teratoma with complete adrenal gland

1 Department of Pathology, Institute of Post Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, India
2 Department of Radiology, Medical College and Hospital, Kolkata, West Bengal, India
3 Department of Neonatal and Fetal Surgery, Institute of Post Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, India

Date of Web Publication15-Feb-2016

Correspondence Address:
Uttara Chatterjee
57/11A, Ballygunje Circular Road, Kolkata - 700 019, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.148690

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 > Abstract 

Sacrococcygeal teratoma is the most common congenital neoplasm in neonates. We came across a post term (42 weeks) newborn baby girl delivered by normal vaginal route. The baby presented with a large soft, cystic mass over the sacrococcygeal region. Radiological examination showed a soft tissue mass with variegated appearance. Complete excision of the mass was done. Histopathological examination revealed the mass to be a mature sacrococcygeal teratoma with the extremely uncommon finding of a complete adrenal gland within the teratoma sac. Sacrococcygeal teratoma is a component of a continuum with other tumors including fetiform teratoma, fetus in fetu, parasitic and conjoint twins.

Keywords: Ectopic adrenal gland, fetiform teratoma, sacrococcygeal teratoma

How to cite this article:
Mandal B, Chatterjee G, Bhattacharya K, Roy D, Das RN, Chatterjee U. Sacrococcygeal teratoma with complete adrenal gland . J Can Res Ther 2015;11:1040

How to cite this URL:
Mandal B, Chatterjee G, Bhattacharya K, Roy D, Das RN, Chatterjee U. Sacrococcygeal teratoma with complete adrenal gland . J Can Res Ther [serial online] 2015 [cited 2020 Feb 19];11:1040. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1040/148690

 > Introduction Top

Sacrococcygeal teratoma is the most common neoplasm in fetus and newborn. [1],[2] They are believed to arise early in gestation from totipotential cells of Hensen's node, remnant of the primitive streak in the coccygeal region. [3] These tumors are composed of multiple types of tissue derived from more than one germ cell layers. Sometimes, mature sacrococcygeal teratomas can be highly differentiated (Fetiform teratoma) showing organoid arrangement of tissues. The diagnosis of fetus in fetu usually means presence of vertebral axis. [4] These lesions form a spectrum of anomalies with no clear-cut distinguishing feature. Here we take the opportunity to report a case of mature sacrococcygeal teratoma in a baby girl with the uncommon finding of presence of complete adrenal gland within the mass.

 > Case report Top

A newborn girl, weighing 3500 g, was delivered at 42 weeks by vaginal delivery. The mother was 35 years old, multipara, and was not a booked case. The baby had a large mass in the sacrococcygeal region [Figure 1].
Figure 1: Photograph showing a large, variegated mass in the sacrococcygeal region

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Routine preoperative investigations were unremarkable. Ultrasonography (USG) showed a variegated solid-cystic mass in the sacrococcygeal region. The mass was predominantly located outside. (Altman Type I) [5] No other associated malformation was present.

Complete excision of the mass was done. She developed post-operative wound infection, meningitis, and left-eye conjunctivitis, which were treated by antibiotics, anticonvulsant, and dressings.

Grossly, the mass was a skin covered sac measuring 7 cm across. On slicing, it had variegated appearance including small cystic areas. In addition, complete adrenal gland was identified. It was pyramidal in shape with cerebriform convolutions on the surface along with a rim of bright yellow cortex and dark tan medulla [Figure 2].
Figure 2: Gross appearance of the variegated mass showing cystic areas (arrowhead) and presence of complete adrenal gland, inset focuses on the pyramidal shaped adrenal gland (arrow) with cerebriform convolutions on the surface along with a rim of bright yellow cortex and dark tan medulla

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Microscopically, the sections showed presence of mature brain tissue, choroid plexus, skin with adenexa, gastrointestinal tract mucosa, and islands of cartilage. The presence of organized adrenal gland bearing both fetal type cortex and medulla was confirmed microscopically. No immature elements were identified. Based on these histopathological features, a diagnosis of mature sacrococcygeal teratoma with the rare presence of complete adrenal gland was made [Figure 3].
Figure 3: (a) Photomicrograph showing cystic spaces lined by columnar epithelium along with islands of cartilage and brain tissue (H and E, ×40). (b) photomicrograph showing complete adrenal gland with organoid arrangement of fetal cortex and medulla (H and E, ×40)

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Post-operative USG showed bilateral normal-looking adrenal glands (with both cortex and medulla) at the upper pole of both kidneys [Figure 4]. She was discharged with well-healed scar. The baby is doing fine with normal developmental milestones in the subsequent follow-up.
Figure 4: Ultrasonography of bilateral renal fossae showing normal right (a) and left (b) adrenals located at upper pole of respective kidneys

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 > Discussion Top

Sacrococcygeal teratomas, although rare, are the most common neoplasm in the newborn with reported incidence of 1/35,000 to 1/40,000 live births. They show marked female preponderance (2-4:1). Earliest description of these tumors in Chaldean Cuneiform tablets dates back to 2000 BCE. [2] The predilection of sacrococcygeal site is probably due to the occurrence of large number of pluripotent cells in the caudal region. [6]

Mature sacrococcygeal teratomas usually have excellent prognosis if completely excised. Antenatal USG is important for diagnosis along with associated features like hydrops, placentomegaly. Prenatal diagnosis with planned cesarean section and immediate excision is the usual management. [7]

The diagnosis of teratoma is usually made by histology, which shows multiple types of tissue derived from one or more of three germ cell layers. Unlike other germ cell tumors, anomalies of chromosome 12 are rare. [8] Thorough histological examination is important to exclude immature component as it adversely affects prognosis.

In the newborn period, majority of sacrococcygeal teratomas are mature. Sometimes, the degree of organization can be so advanced as to take a fetiform appearance. A fetus in fetu is usually diagnosed on the basis of presence of vertebral axis and organoid differentiation. [4] Spencer [9] suggested that a fetus in fetu must have one or more of the following: (1) Enclosed within a distinct sac; (2) partially or completely covered by normal skin; (3) grossly recognizable anatomic parts; (4) attached to host by only a few large blood vessels; (5) located adjacent to one of the sites of attachment of conjoint twins or associated with the neural tube or the gastrointestinal system. On the other hand, a teratoma shows lesser degree of organoid differentiation and has broad site of attachment with multiple vessels. Goldstein et al.,[10] however, have recommended diagnosis of fetus in fetu even in the absence of vertebral axis if the tumor shows highly organoid pattern.

There are also controversies regarding the origin of fetus in fetu. Some advocate the "Identical-twin theory," in which fetus in fetu is a diamniotic, monochorionic, monozygotic twin, others consider it to be an extremely well-organized form of teratoma. [11] Somewhere in between this spectrum lie the highly differentiated sacrococcygeal teratomas (fetiform teratomas) which show more complex organoid structures but fall short of the diagnosis of fetus in fetu. Dealing with these tumors, it is important to keep in mind that teratomas are true neoplasms and are therefore capable of malignant transformation, whereas fetus in fetu is benign.

Many mature and organoid structures have been reported in sacrococcygeal teratomas including intestinal loops, complete with muscle layers and ganglion cells. Limbs, mature brain tissue, thyroid, pancreas, and salivary glands. Presence of hepatic, renal tissue, although rare, has been reported. However, presence of complete adrenal gland has never been reported before. Islands of adrenal cortical rests have been reported very rarely in the wall of the gall bladder, pancreas, mediastinum, lungs, liver, and wall of large intestine. [12] Valdiserri et al. [13] in their large series of sacrococcygeal teratoma have reported presence of adrenal rests in 2 cases. In our case, complete organoid arrangement of fetal adrenal gland including cortex and medulla were clearly identified in the mass.

Here we report a case of mature sacrococcygeal teratoma with the presence of complete adreanal gland, which to our knowledge is the first reported case of this rare association. It is important to realize that a spectrum of anomalies can be seen in neonates ranging from conjoined twins to external parasitic twins to fetus in fetu to "fetiform" teratomas to well-differentiated teratomas depending on the timing of embryological insult and degree of differentiation. Complete surgical excision is the preferred treatment of both fetus in fetu and sacrococcygeal teratoma and these tumors should be kept on close follow-up to detect recurrences or progression to malignancy.

 > Conclusion Top

We report a case of mature sacrococcygeal teratoma in a neonate with the rare finding of complete adrenal gland in it, which to the best of our knowledge, is the first reported case of such an association.

 > References Top

De Backer D, Erpicum P, Philippe P, Demarche M, Otte JB, Schwagten K, et al. Sacrococcygeal teratoma: Results of a retrospective multicentric study in Belgium and Luxembourg. Eur J Pediatr Surg 2001;11:182-5.  Back to cited text no. 1
Tuladhar R, Patole SK, Whitehall JS. Sacrococcygeal teratoma in the perinatal period. Postgrad Med J 2000;76:754-9.  Back to cited text no. 2
Ein SH, Adeyemi SD, Mancer K. Benign sacrococcygeal teratomas in infants and children: A 25 year review. Ann Surg 1980;191:382-4.  Back to cited text no. 3
Lord JM. Intra-abdominal foetus in foetu. J Pathol Bacteriol 1956;72:627-41.  Back to cited text no. 4
Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 1974;9:389-98.  Back to cited text no. 5
Faheem M, Syed HH, Kardam D, Maheshwari V, Khan R, Sharma A. Teratoma of lumbosacral region: A case report. J Med Case Rep 2011;5:370.  Back to cited text no. 6
Okada T, Sasaki F, Cho K, Honda S, Naito S, Hirokata G, et al. Management and outcome in prenatally diagnosed sacrococcygeal teratoma. Pediatr Int 2008;50:576-80.  Back to cited text no. 7
Gurda GT, VandenBussche CJ, Yonescu R, Gonzalez-Roibon N, Ellis CL, Batista DA, et al. Sacrococcygeal teratomas: Clinico-pathological characteristics and isochromosome 12p status. Mod Pathol 2014;27:562-8.  Back to cited text no. 8
Spencer R. Parasitic conjoined twins: External, internal (fetuses in fetu and teratomas), and detached (acardiacs). Clin Anat 2001;14:428-44.  Back to cited text no. 9
Goldstein I, Jakobi P, Groisman G, Itskovitz-Eldor J. Intracranial fetus-in-fetu. Am J Obstet Gynecol 1996;175:1389-90.  Back to cited text no. 10
Ji Y, Chen S, Zhong L, Jiang X, Jin S, Kong F, et al. Fetus in fetu: Two case reports and literature review. BMC Pediatr 2014;14:88.  Back to cited text no. 11
Bal A, Adhya AK, Mahajan JK. Ectopic adrenocortical rest in the wall of the large intestine. Indian J Pathol Microbiol 2009;52:130-1.  Back to cited text no. 12
[PUBMED]  Medknow Journal  
Valdiserri R, Yunis E. Sacrococcygeal teratomas: A review of 68 cases. Cancer 1981;48:217-21.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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