Carcinoid tumour with cystic teratoma in ovary--a case report.
Mature cystic teratomas are benign unilateral tumours, often diagnosed in young females. Teratomas are composed of tissues derived from the three germ layers; ectoderm, mesoderm and endoderm.  The most common sites of occurrence is the ovary.  A malignant tumour that arises in a pre-existing mature teratoma is called teratoma with malignant transformation,  which occurs in 1-3% of all mature teratomas.  Transformation includes thyroid carcinoma, adenocarcinoma and carcinoid tumour. [5,6] Squamous cell carcinoma is the most common form of transformation.  Carcinoid tumours are slow-growing tumours, originating from neuroendocrine cells which are classified into four categories: i) Insular; ii) Trabecular; iii) Strumal; and iv) Mucinous types. [7,8] Carcinoids are most commonly found in the ileum and appendix. While mature cystic teratomas are benign tumours, carcinoid tumours of the ovary are considered to be malignant and are occasionally associated with metastases. [7,8] The overall 5-year survival rate for patients with carcinoids regardless of location is 44-56%.  The presence of regional and distant metastases is associated with a worsening in the prognosis. Hormone and vasoactive amine secretion from the carcinoid tumour cells cause carcinoid syndrome. Serotonin, tachykinins, prostaglandins and histamine are the main secretions of carcinoids. The secretions of these substances cause symptoms like flushing, diarrhoea, bronchial obstruction and heart failure.  Surgery is the main treatment for carcinoid tumours and previous cases of carcinoids arising in mature teratomas of the retroperitoneum and ovary, which were successfully treated by surgical resection have been reported in the literature. [11,12]
A 56 years postmenopausal female came to Krishna Hospital, Karad, on an OPD basis with complaints of pain in abdomen since 1 month. She was second para with both full term vaginal deliveries. The patient was postmenopausal since 10 years. No significant past and family history.
On examination, patient was vitally stable. An adnexal mass was located in right iliac fossa approximately 7 x 7 cm was felt which was mobile, cystic in consistency and regular margins. Cervix and vagina was healthy on per speculum examination. On per vaginal examination uterus size was normal, anteverted and cystic mass felt in right fornix.
All preoperative examinations were normal. Tumour marker CA-125: 8.3 U/mL.
Right ovarian complex lesion measuring 84 x 62 x 78 mm in size, encapsulated with nodular outline suggestive of dermoid cyst.
Patient was posted for laparotomy. Intraoperatively, uterus and left ovary appeared to be normal, while right ovary showed yellowish white surface of cystic consistency of size 79 x 70 x 70 mm. Pan abdominal hysterectomy was performed and the sample sent for HPR.
The HPR showed carcinoid tumour in cystic teratoma: uniform small round cells arranged in solid masses alternating with acinar and ribbons formation separated by fibrous septae with various components like cartilage, dense fibrous and adipose tissue, smooth muscle fibres and areas of ossification.
Malignant transformation is a rare complication of mature cystic teratomas.  Carcinoid tumours are rare and have been presented as case reports previously. [14,15] Carcinoid tumours in ovarian teratomas are considered to be derived from neuroendocrine cells of the gastrointestinal or respiratory epithelium. [12,16] The patient in the present report was postmenopausal. Intraoperatively, uterus and left ovary appeared to be normal, while right ovary showed yellowish white surface of cystic consistency of size 79 x 70 x 70 mm. Pan abdominal hysterectomy was performed.
Carcinoid tumours arising in mature teratomas are tumours with low malignancy potential.  However, the prognosis and clinical behaviour of these tumours have not yet been clarified. 
Without resection of the tumour, the risk of malignant transformation increases with time. All suspected areas in the cyst walls should be sampled in order to define these unusual rare neoplasms. In the present case report, the tumour was in a small area in the cyst wall and completely resected. This case was notable due to the rarity of this occurrence.
This case adds to rare reports in literature of a carcinoid of low malignant potential occurring in a mature cystic teratoma. The treatment for early stage ovarian carcinoid tumours confined to one ovary is surgery alone and excellent outcomes can be expected.
 Prokhorova TA, Harkness LM, Frandsen U, et al. Teratoma formation by human embryonic stem cells is site dependent and enhanced by the presence of matrigel. Stem Cells Dev 2009;18(1):47-54.
 Gatcombe HG, Assikis V, Kooby D, et al. Primary retroperitoneal teratomas: a review of the literature. J Surg Oncol 2004;86(2):107-13.
 Harms D, Zahn S, Gobel U, et al. Pathology and molecular biology of teratomas in childhood and adolescence. Klin Padiatr 2006;218(6):296-302.
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 Hackethal A, Brueggmann D, Bohlmann MK, et al. Squamous-cell carcinoma in mature cystic teratoma of the ovary: systematic review and analysis of published data. Lancet Oncol 2008;9(12):1173-80.
 Kim SM, Choi HS, Byun JS, et al. Mucinous adenocarcinoma and strumal carcinoid tumor arising in one mature cystic teratoma of the ovary with synchronous cervical cancer. J Obstet Gynaecol Res 2003;29(1):28-32.
 Kurman RJ, Ellenson HL, Ronnett BM. Blaustein's pathology of the female genital tract. 6th edn. NY, New York: Springer 2011; p. 883.
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 Flam F, Silfversward C. Combination of granulosa cell tumour and carcinoid in an imitation of appendix vermicularis within a mature teratoma--a unique case. Eur J Obstet Gynecol Reprod Biol 1994;56(2):139-42.
 Chatzipantelis P, Mavrogiorgis A, Kairi-Vassilatou E, et al. Ovarian neoplasm composed of an insular carcinoid tumor and a borderline mucinous cystadenoma arising in a mature cystic teratoma: a case report. Eur J Gynaecol Oncol 2006;27(6):636-7.
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Sanjaykumar Patil (1), Sonal Phadtare (2)
(1) Professor, Department of Obstetrics and Gynaecology, Krishna Institute of Medical Sciences, Karad.
(2) Postgraduate Student, Department of Obstetrics and Gynaecology, Krishna Institute of Medical Sciences, Karad.
Financial or Other, Competing Interest: None.
Submission 01-02-2017, Peer Review 03-03-2017, Acceptance 09-03-2017, Published 16-03-2017.
Dr. Sanjaykumar Patil, Krishna Hospital, Karad.
Capiton: Figure 1. USG
Caption: Figure 2. Specimen
Caption: Figure 3. Histopathological Slide
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|Title Annotation:||Case Report|
|Author:||Patil, Sanjaykumar; Phadtare, Sonal|
|Publication:||Journal of Evolution of Medical and Dental Sciences|
|Article Type:||Case study|
|Date:||Mar 16, 2017|
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