Adeno-squamous carcinoma arising in mature cystic teratoma: A rare case report.
CASE REPORT: A 36 years old female presented with the complaints of abdominal pain, abdominal fullness and difficulty in periods since 6 months. She does not have a known medical history and family history of cancer. Physical examination of lower abdomen revealed fullness and feeling of a mass. CECT abdomen showed a well-defined cystic lesion of size 13.5x13cm in left adnexal region extending upto umbilicus. A heterogeneously enhancing nodule of sized 3x2.5cm seen in anterior aspect of lesion. Comple blood count, Liver function, Kidney function test values were normal.
Tumour markers CA-125, CA 19.9, CEA were normal too. Patient underwent oophorectomy. Grossly we received ovarian cyst measuring 13x11x10cm.Cut surface shows multiple cyst like spaces filled with cheesy material and bunch of hair. (Fig. 1, 2) Wall is thickened at places (Fig. 2 indicated by arrow) and shows grayish white firm area measuring 0.8x0.5cm.Microscopically sections from the thickened area show features of adenosquamous carcinoma arising in mature cystic teratoma. (Fig. 3,4,5,6)
DISCUSSION: Malignant transformation of MCTs may arise from any of three germ cell layers present in the teratoma, with an average frequency of 1-2%.  Age and size are predictors of malignant transformation in benign teratoma. It is of great importance to know that MCTs arising in patients older than 45 years old sustain a higher suspicion of malignancy.  Malignant transformed mature cystic teratomas have a larger size (Mean size of 15 cm) compared with MCTs (mean size of 6-9 cm). 
Symptoms at presentation are variable in both diagnoses with some patients presenting with acute abdominal pain and others with constitutional symptoms such as fatigue, urinary symptoms and anorexia/weight loss.  In our case, woman's age was 36 years old and presented with the complains of abdominal pain and it is worth to mention her premenopausal status. Although tumor markers may be raised in patients with squamous cell carcinoma arising from MCT, it is difficult to use tumor markers to distinguish between MCT and squamous cell carcinoma arising from an MCT since tumor markers can also be elevated with MCT. 
For example, squamous cell antigen (SCC) levels were found to be significantly higher in patients with squamous cell carcinoma arising from MCT than with MCT alone. However, mean levels in squamous cell carcinomas are lower than in patients with adenocarcinomas, and cannot predict the diagnosis preoperatively.  CA19.9 is another tumor marker found to be significantly higher in patients with squamous cell carcinoma arising from MCT than with MCT alone, however it is also a difficult marker to use in preoperative screening since the mean levels of CA 19.9 are found to be elevated in patients with MCT alone. 
In our case all tumour markers were in normal range. Radiologically, mature teratomas may demonstrate a broad spectrum of findings ranging from a purely cystic mass, a fat-containing mass or a heterogeneous soft tissue mass. Classic diagnostic findings for MCT include fat attenuation within a cyst that possibly also contains calcification.  Imaging features concerning for malignant transformation include thick walls, enhancing solid components or papillary projections within the cyst, peritoneal deposits or lymphadenopathy. In our case CECT abdomen showed a well-defined cystic lesion of size 13.5x13cm with a heterogeneously enhancing nodule of sized 3x2.5cm seen in anterior aspect of lesion.
As a result of the rarity of adenoaquamous carcinomaarising in MCT, there is no standard treatment and most often patients are treated in the same way with those patients as with epithelial ovarian cancer. Multiple case series recommend surgery including total hysterectomy, bilateral salpingo-oophorectomy, omentectomy and pelvic-paraortic lymph node dissection with further platinum-based agent chemotherapy [2,12] The role of radiotherapy still remains unclear. [2,12] In our case, after the final pathologic specimen result, patient proceeded to a
second surgery including total hysterectomy, left salpingo-oophorectomy, omentectomy and pelvic node dissection.
CONCLUSION: A rare and unusual disorder is adenosquamous carcinomaarising in an MCT. As an exceedingly rare disease there is no standard of therapy, with most cases progressing to surgery followed by chemotherapy.
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Shalini Shukla (1), Sanjay Nigam (2), Jayant Rai (3), Parul Joshi (4), Neha Ahuja (5)
(1.) Shalini Shukla
(2.) Sanjay Nigam
(3.) Jayant Rai
(4.) Parul Joshi
(5.) Neha Ahuja
PARTICULARS OF CONTRIBUTORS:
(1.) Lecturer, Department of Pathology, GSVM Medical College, Kanpur.
(2.) Professor, Department of Pathology, Rama Medical College and Research Centre, Kanpur.
(3.) 2nd Year Junior Resident, Department of Pharmacology, GMC, Surat.
(4.) Assistant Professor, Department of Pathology, Rama Medical College and Research Centre, Kanpur.
(5.) Consultant Pathologist, Paliwal Diagnostic Private Limited.
FINANCIAL OR OTHER COMPETING INTERESTS: None
NAME ADDRESS EMAIL ID OF THE CORRESPONDING AUTHOR:
Dr. Sanjay Nigam, Professor of Pathology, Rama Medical College and Research Centre, Kanpur.
Date of Submission: 23/9/2015. Date of Peer Review: 24/9/2015. Date of Acceptance: 08/10/2015. Date of Publishing: 19/10/2015.
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|Title Annotation:||CASE REPORT|
|Author:||Shukla, Shalini; Nigam, Sanjay; Rai, Jayant; Joshi, Parul; Ahuja, Neha|
|Publication:||Journal of Evolution of Medical and Dental Sciences|
|Date:||Oct 19, 2015|
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