Presacral region is defined as potential space delineated by the rectum anteriorly, sacrum posteriorly, peritoneal reflection superiorly, and perineal muscles inferiorly. Tumors within the presacral space are uncommon with an incidence of 1:40,000. Presacral teratomas in adults are very rare. We, herein, present a case of 39‑year‑old male who was admitted with chief complaint of per rectal bleed. A large presacral mass was present which on histopathological examination was found to be immature teratoma with neuroendocrine differentiation

Kaur, Arshpreet

Kaur, Kanwardeep

Kaur, Manmeet

Nibhoria, Sarita

English

Atharva Scientific Publications (E-Jounals)

Vol 3 | Issue 4 | Oct - Dec 2017 Indian J Case Reports 273
Case Report
Presacral immature teratoma with carcinoid in a young adult male: A rare case 
presentation
Kanwardeep Kaur, Sarita Nibhoria, Manmeet Kaur, Arshpreet Kaur
From Department of Pathology, Guru Gobind Singh Medical College, Faridkot, Punjab, India
Correspondence to: Dr. Arshpreet Kaur, Department of Pathology, Guru Gobind Singh Medical College, Faridkot, Punjab, India. 
E‑mail: kaurarshpreet2090@gmail.com
Received ‑ 06 September 2017 Initial Review ‑ 05 October 2017 Published Online ‑ 10 November 2017
Presacral region is defined as potential space delineated by the rectum anteriorly, sacrum posteriorly, peritoneal reflection superiorly, and perineal muscles inferiorly [1]. 
Tumors within the presacral space are uncommon with 
an incidence of 1:40,000 [2]. The most common tumor in 
presacral space is chordomas [3]. The tumors in presacral 
space may arise within tail gut cysts and teratomas suggesting 
the congenital nature of these tumors [3,4]. Teratomas within 
the presacral space most commonly occur in infancy and 
have an incidence of 1 in 30,000–43,000 live births with a 
3–4:1 female‑to‑male ratio [2,5,6]. Rarely, do presacral 
teratomas present in adulthood. We, herein, present a case of 
39‑year-old male who was admitted with chief complaint of 
per rectal bleed. A large presacral mass was present which 
on histopathological examination was found to be immature 
teratoma with neuroendocrine differentiation.
CASE REPORT
A 39‑year‑old male was hospitalized with the chief complaint 
of constipation and per rectal bleeding for past 4–5 months. 
These symptoms were on and off initially but now progressed. 
The patient had not taken any treatment of the same in the 
past. On examination, the patient was bit pale with the normal 
vital signs. On per rectal examination, a large firm, nontender 
mass was found between the rectum and sacrum. A complete 
hemogram was performed which showed a hemoglobin of 5.1 g/
dl with all other parameters being normal. Rest of the routine 
investigations was normal. Magnetic resonance imaging was 
done which showed the presence of a large well defined solid 
cystic mass in the presacral space with solid component showing 
intense heterogeneous enhancement (Fig. 1). The mass was seen 
compressing and displacing the rectum anteriorly (Fig. 2). The 
possibility of malignant teratoma was being suggested.
Complete excision of the mass was done and sent for 
histopathological examination. Grossly, the mass was gray white to 
gray brown measuring 8 cm × 7 cm × 3.5 cm. Cut section of this 
mass showed partial solid areas with multiple cysts ranging in size 
from 0.5 to 2 cm in diameter. These cysts were filled with pultaceous 
material (Fig. 3). Histopathological examination revealed the 
presence of immature teratoma with neuroendocrine differentiation 
possibly carcinoid (Figs. 4‑6). An immunohistochemistry panel 
was applied which showed immunoreactivity for neuron‑specific 
enolase. However, glial fibrillary acidic protein was found to be 
negative (Fig. 7). Diagnosis of immature teratoma with carcinoid 
was made and the patient was advised for complete surgical excision.
DISCUSSION
Presacral space contains multiple embryological remnants and is a 
site for the development of various types of tumors. Various tumors 
or tumor‑like lesions including teratoma, chordoma, myxopapillary 
ependymal, paraganglioma, schwannoma, liposarcoma, tail gut 
cyst, and metastatic tumor can occur in this area with increased 
incidence. Chordomas are the most common among them [3]. 
Presacral teratomas in adults are very rare. The first reported case 
of a presacral teratoma in an adult was published by Emmerich in 
1847 and was included as one of six cases reviewed by Kiderlen, in 
1899 [7]. Wishnia et al. in their study, in 2008, demonstrated fewer 
than 120 reported cases of isolated adult presacral teratoma [8]. Of 
those patients whose sex was reported (92 of 104 patients), 70% 
were female and 30% were male.
ABSTRACT
Presacral region is defined as potential space delineated by the rectum anteriorly, sacrum posteriorly, peritoneal reflection superiorly, 
and perineal muscles inferiorly. Tumors within the presacral space are uncommon with an incidence of 1:40,000. Presacral 
teratomas in adults are very rare. We, herein, present a case of 39‑year‑old male who was admitted with chief complaint of per 
rectal bleed. A large presacral mass was present which on histopathological examination was found to be immature teratoma with 
neuroendocrine differentiation.
Key words: Presacral, Immature, Teratoma, Tumors
Kaur et al. Presacral  immature teratoma with carcinoid in a young adult male: A rare case presentation
Vol 3 | Issue 4 | Oct - Dec 2017 Indian J Case Reports 274
Macroscopically, teratomas are partially cystic masses filled 
with gelatinous fluid or keratin. Microscopically, they contain a 
variety of cell types derived from more than one germ layer [9]. 
Teratomas can be classified into three histologic categories: 
Mature, immature, and malignant. Mature teratomas have benign 
epithelial‑lined cysts, mature cartilage, and/or muscle. Immature 
teratomas contain primitive mesoderm, endoderm, or ectoderm 
admixed with the mature elements. Malignant teratomas contain 
frank malignant tissue which may or may not be of germ cell 
origin [6]. Carcinoid tumors within the presacral space are rare 
Figure 1: Large well defined solid cystic mass in the presacral space 
with solid component showing intense heterogenous enhancement
Figure 2: This mass was seen compressing and displacing the rectum 
anteriorly
Figure 3: Grossly, the mass was gray white to gray brown measuring 
8 cm x 7 cm x 3.5 cm. Cut section showed partial solid areas with 
multiple cysts ranging in size from 0.5 to 2 cm in diameter
Figure 4: Low power view of the tumor arranged in trabecular 
pattern with the presence of myxoid stroma
Figure 5: High power view showing individual tumor cells having 
round to oval nuclei having granular chromatin and mild amount 
of cytoplasm
Figure 6: Squamous component of the teratoma is seen as lining of 
stratified squamous epithelium
Kaur et al. Presacral  immature teratoma with carcinoid in a young adult male: A rare case presentation
Vol 3 | Issue 4 | Oct - Dec 2017 Indian J Case Reports 275
and most often represent the direct extension or metastatic spread 
from adjacent rectal carcinoids [2,10]. Dujardin et al. found 
20 cases of primary neuroendocrine carcinoma in the presacral 
space reported in the literature in 2009 [11]. Spada et al., in 2010, 
stated that only 28 cases of carcinoid tumors of the presacral 
region are described in the medical literature and of these 14 were 
associated with tail gut cysts. The rest was either associated with 
teratomas or with no other peculiar anomalies [12].
These tumors are managed by surgical resection. The 
prognosis of mature teratoma is good while malignant teratoma 
is poor with a higher tendency to recur and metastasize [5,6]. The 
5 years survival rate for presacral neuroendocrine carcinoma with 
metastasis has been reported to be 20–30% [11].
CONCLUSIONS
Carcinoid tumors in presacral region are rare. Malignant 
components within a teratoma need to be differentiated from 
Figure 7: Immunohistochemistry showing neuron-specific enolase 
positivity
primary and metastatic tumors. These tumors should be included 
in the differential diagnosis of presacral mass and close follow‑up 
is required for early diagnosis and management of recurrence or 
metastasis.
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Funding: None; Conflict of Interest: None Stated.
How to cite this article: Kaur K, Nibhoria S, Kaur M, Kaur A. Presacral 
immature teratoma with carcinoid in a young adult male: A rare case 
presentation. Indian J Case Reports. 2017;3(4):273‑275.


Presacral immature teratoma with carcinoid in a young adult male: A rare case presentation

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Presacral immature teratoma with carcinoid in a young adult male: A rare case presentation

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