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Abstract 

Primary retroperitoneal teratoma is a rare entity in adults. It has a distinctive imaging

appearance. We report a case of a 27-years-old patient who came to our hospital with
the complaint of lower abdominal asymmetrical swelling. Radiological work-up were
done which showed feature of retroperitoneal teratoma. Exploratory laparotomy with
tumor resection was performed. Pathological examination revealed a benign cystic
mature teratoma. The patient is doing well at follow-up. This report reviews the literature
and addresses the incidence, etiology, and treatment of primary extra-gonadal germ cell
tumor mature teratoma.

Introduction

Teratoma is uncommon neoplasm consist of mixed dermal elements derived from all
three germ cell layers. Majority of teratoma present congenitally in the sacrococcygeal
region, within the ovaries of adolescent females and within the testes of young men,
they have been identified throughout the body. Extra-gonadal teratoma tend to occur in
midline structures as the anterior mediastinum, retro-peritoneum, sacrococcygeal
region, and pineal gland. Retroperitoneal teratoma represents only 1–11% of primary
retroperitoneal tumors. Incidence is bimodal with peaks in the first 6 months of life and
in early adulthood. Due to their location, they are usually identified only after they have
grown to huge proportions. Surgical resection remains the mainstay of therapy and is
required for definitive diagnosis. This article reviews the literature on the histopathology,
classification, the presentation, diagnosis, and management of retroperitoneal teratoma.

Case report

27 years old male presented with lower abdominal swelling for 8 to 9 month ago, initially
associated with discomfort. There was no history of significant weight loss. He used to
work as a driver and smoked 10 packs year. General physical examination was
unremarkable. Abdominal examination revealed lower non tender, non compressible,
non reducible abdominal mass, measuring 12 x 12 cm with overlying smooth surface, it
was dull on percussion. There was no other palpable regional and non regional lymph
node in the body. DRE showed small prostate with smooth surface and mobile mucosa.
There was smooth surface mass extending from anterior rectal wall above the prostate.
Rest of systemic examination was normal.

Ultrasound showed large hypo-echoic mass below the umbilical region measuring
17x10 cm. Initial blood investigation including CBC, urea, creatinine and electrolyte was
normal. CT scan whole abdomen showed large mass which was identified in pelvis
arising from left seminal vesicle demonstrating variable attenuation values with fat fluid
level causing extrinsic compression and displacement of viscera and also causing
compression to distal ureter resulting obstructive uropathy. See figure 1.

Figure 1: Well circumcised cystic non enhancing mass showing calcification (tooth)
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Tooth

Exploratory laparotomy was performed showed large firm cystic mass arising from
pelvis extending up to umbilicus which causing extrinsic pressure to bowel and urinary
bladder invasion was limited seminal vesicle. Structure like teeth and hair were arising
from the cystic mass. The cystic mass was excised. Postoperative course was
unremarkable.

Histopathology report showed mature cystic teratoma (dermoid cyst). There was no
immature tissue in it. Adherent unremarkable seminal vesicle was seen. There was no
granuloma or malignancy seen. See figure 2.

Figure2: Mature Cystic Teratoma showing Squamous Epithelium, Glial Tissue, Fibroadipose Tissue,
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Bone marrow and Cartilage

Discussion

Germ cell tumors (GCTs) can be broadly classified into two main categories;
seminomatous and non-seminomatous GCTs. Teratomas belong to non-seminomatous
GCTs and represent the most common form of all GCTs.

Teratomas are encapsulated neoplasm composed of multiple parenchymal tissues that

are derived from more than one germ cell layer (ectoderm, mesoderm, and endoderm.
Teratoma arises from uncontrolled proliferation of pluripotent germ cells and
embryonal cells. Teratoma of germ cell source can be congenital or acquired and
are usually found in gonads (testes and ovaries). In contrast, teratoma of
embryonic cell sources are always congenital and are usually found in
extragonadal locations such as intracranial, cervical, retroperitoneal, mediastinal,
and sacrococcygeal sites.

Teratoma can be classified into solid, cystic, or mixed teratoma. Solid teratoma
lack organization and contain only parenchymal tissues. Cystic teratoma contain
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only sacs of fluid, semi-fluid, or fat, whereas mixed teratoma contain both solid
and cystic components. According to the epithelial lining and dermal contents of
tumor, teratoma can be classified into epidermoid, dermoid, and teratoid
teratoma (cysts). Epidermoid teratoma is lined by stratified squamous epithelium
and lack dermal contents. Dermoid teratoma is mostly lined by stratified
squamous epithelium and contains various dermal contents such as hair, sweat,
and sebaceous glands. Teratoid teratoma are mostly lined by respiratory
columnar epithelium and contain sebum. In addition, depending to the degree of
tumor maturation, teratoma can be classified into mature and immature teratoma.
They are highly variable on histology and can be solid, cystic, or mixed. They
contain different types of parenchymal tissues that are well differentiated. Mature
cystic may have partially to completely well-developed organ systems. On the
contrary, immature teratoma is histologically solid teratoma and contains
immature (undifferentiated/undeveloped) parenchymal tissues and can be
possibly benign, possibly malignant. They are more common among males.
Some mature (benign) and immature (possibly benign or possibly malignant)
teratoma has an increased tendency to convert into malignant teratoma and have
increased propensity to metastasize.

Most of retroperitoneal teratoma is secondary neoplasm. Primary retroperitoneal

teratoma is extremely unusual neoplasm accounting for approximately 1–11% of
all primary retroperitoneal neoplasm and typically occurs in neonates, infants,
and children age groups. In adults, these neoplasm are extremely rare and
present in the third or fourth decade.

Teratomas can be diagnosed based on high index of suspicion, routine

laboratory, and radiographic investigations. Clinical presentations may include
nonspecific, abdominal/flank/back pain, obstructive gastrointestinal and
genitourinary symptoms, as well as lower limb/genital swelling due to lymphatic
obstruction. They can rarely present with complications as secondary infections
(abscess formation), traumatic rupture leading to acute peritonitis or malignant
transformations. Midline teratoma masses can be easily detected clinically.
Radiographic investigations play valuable role in diagnosis of teratoma. Plain
radiographs can identify calcified elements in 62% of cases whereas ultrasound
(US) can greatly differentiate between cystic and solid elements. Computed
tomography (CT) scans can help to distinguish between fat and calcified masses.
On the contrary, magnetic resonance imaging (MRI) scans can offer better
resolution of soft tissues. However, definitive diagnosis of teratoma demands a
histopathological evaluation. A testicular ultrasound (US) should be done to rule
out potential coexisting germ cell tumors as approximately 50% of men with
retroperitoneal teratoma have testicular carcinoma at diagnosis.
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Surgical excision of benign (mature) teratoma is required for a definitive

diagnosis by histopathological examination and remains the mainstay of
treatment. Prognosis is fortunately excellent after complete surgical excision.
Teratomas are largely resistant to radio- and chemotherapy. Adjuvant radio- and
chemotherapy are used only if malignant features of germ cell tumors are
identified on histopathological examination.

Conclusion

Primary retroperitoneal teratoma is uncommon and its occurrence in an adult is

exceptionally rare. Histopathological examination of the resected tumor warrants
a definitive diagnosis. Surgical excision of mature teratoma remains the mainstay
of treatment with an excellent five-year survival rate of nearly 100%.

Reference

1. Bhatti A, Al-Hindi H, Azzam A, Amin T, Abu-Zaid A. Mature (Benign) Cystic Retroperitoneal

Teratoma Involving the Left Adrenal Gland in a 22-Year-Old Male: A Case Report and Literature Review.
Case reports in oncological medicine. 2013;2013.
2. Gatcombe HG, Assikis V, Kooby D, Johnstone PA. Primary retroperitoneal teratomas: a review of
the literature. Journal of surgical oncology. 2004;86(2):107-13.
3. Mathur P, Lopez-Viego MA, Howell M. Giant primary retroperitoneal teratoma in an adult: a
case report. Case reports in medicine. 2010;2010.
4. McKenney JK, Heerema-McKenney A, Rouse RV. Extragonadal germ cell tumors: a review with
emphasis on pathologic features, clinical prognostic variables, and differential diagnostic considerations.
Advances in anatomic pathology. 2007;14(2):69-92.
5. Grosfeld JL, Billmire DF. Teratomas in infancy and childhood. Current problems in cancer.
1985;9(9):1-53.
6. Harms D, Zahn S, Göbel U, Schneider D. Pathology and molecular biology of teratomas in
childhood and adolescence. Klinische Pädiatrie. 2006;218(06):296-302.
7. Bedri S, Erfanian K, Schwaitzberg S, Tischler AS. Mature cystic teratoma involving adrenal gland.
Endocrine pathology. 2002;13(1):59-64.
8. Li Y, Yang B, Song L, Yan D. Mature teratoma of the spinal cord in adults: An unusual case.
Oncology letters. 2013;6(4):942-6.
9. Taori K, Rathod J, Deshmukh A, Sheorain VS, Jawale R, Sanyal R, et al. Primary extragonadal
retroperitoneal teratoma in an adult. Brit J Radiol. 2014;79(946).