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  Table of Contents    
CASE REPORT  
Year : 2014  |  Volume : 57  |  Issue : 1  |  Page : 113-115
Pelvic mature cystic teratoma with neuroendocrine carcinoma: Report of a rare association and review of literature


1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Surgery, All India Institute of Medical Sciences, New Delhi, India
3 Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication17-Apr-2014
 

   Abstract 

Extra-gonadal malignant transformation of teratoma is rare and there are only a few reports available citing malignancy arising in the non-germ cell components. We hereby report a case of a 35-year-old female, who presented with lower backache with a radiologically identifiable mass lesion in the pre-sacral region. Clinical and radiological findings suggested the possibility of a cystic teratoma. Histopathological examination and relevant immunohistochemical tests detected a mature cystic teratoma with features of a grade 2 neuroendocrine tumor in it. Like the index case, most of the previously reported cases of teratoma with malignant transformation of the somatic components were found in extra-gonadal site. This case is being reported to emphasize that any extra-gonadal mass in reproductive age group, even if it appears radiologically and per-operatively benign, must be subjected to histopathological examination to rule out possibility of malignant transformation of the germ cell or non-germ cell components.

Keywords: Extragonadal, malignant transformation, mature cystic teratoma, neuroendocrine tumor, somatic component

How to cite this article:
Sable MN, Nath D, Chumbar S, Das CJ, Priyadarshini P, Kaur K, Ranjan R, Gupta SD, Das P. Pelvic mature cystic teratoma with neuroendocrine carcinoma: Report of a rare association and review of literature. Indian J Pathol Microbiol 2014;57:113-5

How to cite this URL:
Sable MN, Nath D, Chumbar S, Das CJ, Priyadarshini P, Kaur K, Ranjan R, Gupta SD, Das P. Pelvic mature cystic teratoma with neuroendocrine carcinoma: Report of a rare association and review of literature. Indian J Pathol Microbiol [serial online] 2014 [cited 2020 Apr 4];57:113-5. Available from: http://www.ijpmonline.org/text.asp?2014/57/1/113/130916



   Introduction Top


Teratoma is a germ cell neoplasm comprising of tissues representing more than one type of embryonic layers, e.g., ectoderm, mesoderm and endoderm. Mature cystic teratoma (MCT) is most common germ cell tumor both in gonads and at extra-gonadal sites (1-5% of all teratomas). [1] Malignant transformation in a MCT is rare. Here, we describe a case of grade 2 neuroendocrine tumor arising in an extra-gonadal pelvic MCT. Though, malignancies arising from the somatic components in a MCT have been described in both gonads as well as in extra-gonadal regions, including retroperitoneum, mediastinum and pelvis, to the best of our knowledge only a few neuroendocrine tumors have been reported originating in a mature pelvic teratoma.


   Case Report Top


This was a case report of a 35-year-old female patient who presented with the complaints of lower backache, which was dull aching, intermittent in nature and gradually progressive over a period of 4 years. There were no complaints of associated lower limb weakness, altered bowel or bladder habits. There was past history of sacrococcygeal tumor excision at the age of 2 years. There was no significant obstetric history. On clinical examination, she was afebrile with normal motor and sensory nervous systems. Local examination showed a scar mark over the previous site of sacral surgery. On per rectal examination, a smooth lobulated tumor can be felt posterior to the rectum, the tumor was free from rectum and the upper limit of the lesion could not be reached. She underwent radiological examination and subsequent exploratory laparotomy.

Radiological findings

Computed tomography (CT) showed a large (10 cm × 8 cm × 6 cm), well-defined, encapsulated, complex cystic lesion in the pre-sacral and pre-coccygeal space extending to the right ischio-rectal area. The mass showed foci of peripheral curvilinear calcification and was displacing the rectum anteriorly and to the left. Bilateral ovaries were separately visualized and were within the normal limits. Other pelvic organs including rectum and uterus were unremarkable [Figure 1]. Magnetic resonance imaging showed a large mass with predominant fat and mutiloculated cystic component in pre-sacral location extending into the ischio-rectal fossa with a provisional diagnosis of teratoma. Bone scan showed no evidence of skeletal metastasis.

A lower midline laparotomy was performed. At operation, a large vascular cystic mass measuring 12 cm × 10 cm × 3.5 cm was seen posterior to the rectum. The mass was removed and due to diffuse ooze packing was done and the same was removed after 48 h. Bilateral ovaries were normal. The patient was discharged after a short hospital stay and had a smooth post-operative recovery.

Histopathological findings

Grossly, the tumor was well circumscribed and measured 12 cm × 9 cm × 3.5 cm in size. Cut surface showed multiple solid and cystic areas of varying sizes, the largest one measured 7 cm × 4 cm × 2 cm and was filled with pultaceous material. The solid areas showed yellowish islands with intermittent grey white nodules and a few necrotic foci [Figure 2]a.

Multiple sections were taken from every 2 cm of the tumor, which showed a multi-cystic tumor focally lined by partially ulcerated stratified squamous epithelium with intra-cystic accumulation of flakes of keratin. The ulcerated area showed hemosiderin laden macrophages and giant cell reactions. The other cysts show respiratory ciliated columnar epithelium, intestinal epithelium and mucus secreting columnar cell lining. There were thick smooth muscle fascicles noted in the cyst wall, admixed with foci of mature adipose tissue. Focal lymphocytic aggregates were seen. Immature cartilage or neural elements were not identified. Other components of mixed germ cell tumor were not noted. All these features were consistent with a MCT. In addition, there were lobules and cords of moderately pleomorphic cells with salt and pepper chromatin with a high mitotic activity counting around 18/10 high power fields. Focally these tumor cells were seen to ulcerate through the overlying stratified squamous epithelium of the keratinous cysts. On immunohistochemical examination, the tumor cells were diffusely and strongly positive for cytokeratin and synaptophysin. Ki67 labeling index was around 2% in the highest proliferating areas. A diagnosis of extragonadal MCT with neuroendocrine tumor/carcinoma of intermediate malignant potential (G2, WHO grading system for neuroendocrine tumors 2010) was offered in view of presence of high mitotic activity [Figure 2]b.

Post-operatively the patient was referred to the oncology department for chemotherapy.


   Discussion Top


MCTs are derived from primordial gem cells and contain cellular elements derived from all three germ cell layers. They are seen in all age groups, predominantly in the third and fourth decades of life. These tumors are usually found in the gonads, sacrococcygeal region and mediastinum. [1]

In the 4 th week of intrauterine fetal life, the germ cells migrate to the midline for development of gonads. During this migration, some of the cells may be retained in the tract and mature later in life to produce a teratoma. Thus, most of the extragonadal teratomas are usually seen in the midline, as in the anterior mediastinum, retroperitoneum and intracranial region. Primary retroperitoneal teratomas comprises 1-5% of all the retroperitoneal tumors. [1]

Malignant transformation in MCTs is noted in about 1-3% cases, with examples both in the gonads or extra-gonadal sites (25%). [1] Most of these malignancies arise from the embryonic tissue resulting in germinoma, choriocarcinoma, yolk sac tumor or embryonal carcinomas. Malignant transformation of the somatic component is less common. Among the latter, squamous cell carcinoma accounts for 70-88% of cases, followed by adenocarcinomas. [2] Other very rare examples of reported somatic malignancies arising in a MCT includes: Adeno-squamous carcinoma, sebaceous carcinoma, malignant melanoma and rarely sarcomas. [3],[4],[5] Neuroendocrine tumor/carcinoma arising within a MCT is extremely rare. There are only five examples of MCT with malignant neuroendocrine transformation in English literature, situated mainly in the retroperitoneum and pelvis [Table 1]. [6],[7],[8],[9],[10]
Table 1: Details of the extra-gonadal teratoma with neuroendocrine tumors

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All these tumors had been identified in women, between the age group of 22-68 years (mean age 45 years) with most common complaint of chronic abdominal pain and fullness as these tumor usually measures > 5 cm in diameter. In one case, the tumor was detected incidentally and was associated with Currarino syndrome. [6] In the index case, the patient presented with gradually increasing lower backache due to compression on the adjacent structures. Similar radiological findings were noted in all cases, with the primary lesion presented as a complex solid and cystic tumor.

In the previously reported cases the secondary neuroendocrine tumor were mainly noted in and around the bronchial and gatro-intestinal epithelium. [6],[7],[8],[9],[10] In the present case, the neuroendocrine tumor was also seen to arise adjacent close to the bronchial epithelium, with extension and infiltration in the cysts lined by stratified squamous epithelium [Figure 2]c and d.
Figure 1: Saggital unenhanced computed tomography scan image showing a large soft tissue mass (asterisk) in pre-sacral region containing fat

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Figure 2: A solid cystic tumor with yellowish areas (arrow) (a, gross). A mature cystic teratoma showed squamous (b, H and E, 40) and ciliated columnar epithelial linings (c, H and E, 40; and d, H and E, 100]. An infiltrating tumor with cells having salt and pepper chromatin was seen arising in the peri-bronchial region (b and d). Frequent mitotic figures were noted (arrow) (e, H and E, 200]. Immunohistochemical (IHC) stains for cytokeratin (f, IHC [cytokeratin] 100), synaptophysin (g, IHC [synaptophysin] 100) and Ki67 (arrow) (h, IHC [Ki67] 100] have been shown

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Classically, like that of the index case, due to the benign nature of these tumors, they are treated by surgical excision. The overall disease free survival after excision depending on the success of the surgical excision and if there is no capsular breach. [6],[7],[8],[9],[10] The exact role of chemotherapy in such cases has not been fully established. Our case has also been scheduled for adjuvant chemotherapy.

Therefore, to summarize, malignant transformation of the non-germ cell/somatic component of a MCT is rare with only an occasional examples in literature. Such transformations have been mostly noted in the reproductive age group and in extra-gonadal locations. The index case emphasizes the need of histological examination in all pelvic tumors in women of reproductive age even if radiological and per-operative findings are not suspicious for a malignant transformation.

 
   References Top

1.Genadry R, Parmley T, Woodruff JD. Secondary malignancies in benign cystic teratomas. Gynecol Oncol 1979;8:246-51.  Back to cited text no. 1
[PUBMED]    
2.Kikkawa F, Ishikawa H, Tamakoshi K, Nawa A, Suganuma N, Tomoda Y. Squamous cell carcinoma arising from mature cystic teratoma of the ovary: A clinicopathologic analysis. Obstet Gynecol 1997;89:1017-22.  Back to cited text no. 2
    
3.Vartanian RK, McRae B, Hessler RB. Sebaceous carcinoma arising in a mature cystic teratoma of the ovary. Int J Gynecol Pathol 2002;21:418-21.  Back to cited text no. 3
    
4.Vimla N, Kumar L, Thulkar S, Bal S, Dawar R. Primary malignant melanoma in ovarian cystic teratoma. Gynecol Oncol 2001;82:380-3.  Back to cited text no. 4
    
5.Climie AR, Heath LP. Malignant degeneration of benign cystic teratomas of the ovary. Review of the literature and report of a chondrosarcoma and carcinoid tumor. Cancer 1968;22:824-32.  Back to cited text no. 5
[PUBMED]    
6.Yamasaki T, Yagihashi Y, Shirahase T, Hashimura T, Watanabe C. Primary carcinoid tumor arising in a retroperitoneal mature teratoma in an adult. Int J Urol 2004;11:912-5.  Back to cited text no. 6
    
7.Arazi M, Toy H, Tavli L. Primary neuroendocrine carcinoma arising within a mature sacrococcygeal teratoma. Orthopedics 2007;30:878-9.  Back to cited text no. 7
    
8.Pendlimari R, Leonard D, Dozois EJ. Rare malignant neuroendocrine transformation of a presacral teratoma in patient with Currarino syndrome. Int J Colorectal Dis 2010;25:1383-4.  Back to cited text no. 8
[PUBMED]    
9.Scott AL, Abbassi-Ghadi N, Archer CM, Swamy R, Gupta S. Neuroendocrine carcinoma arising within a retroperitoneal mature teratoma. Ann R Coll Surg Engl 2010;92:W5-8.  Back to cited text no. 9
    
10.Michael K, Kavin GS, Alan K, Ernesto PM, James S. Malignant carcinoid arising within a mature cystic teratoma of the pelvis. J Solid Tumors 2012;2:56-8.  Back to cited text no. 10
    

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Correspondence Address:
Prasenjit Das
Department of Pathology, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.130916

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