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  Table of Contents    
CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 3  |  Page : 440-442
Metastasizing uterine mullerian adenosarcoma in a young female: Diagnostic enigma


1 Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
2 Department of Surgical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
3 Department of Radiology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
4 Department of Medical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India

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Date of Web Publication13-Jul-2018
 

   Abstract 


Uterine adenosarcomas are uncommon tumors. It is a biphasic tumor with both epithelial and mesenchymal component. The epithelial component is benign in nature, and the mesenchymal component is malignant. Metastasis is rare in adenosarcoma. We report a case of adenosarcoma with lymph nodal metastasis. A 20-year-old female presented with history of per vaginal bleeding for 1 month. Per vaginal examination revealed a fungating mass protruding through the cervical os. Ultrasonography and magnetic resonance imaging showed a large intrauterine mass. Biopsy of the mass done at an outside hospital was reported as rhabdomyosarcoma. Hence, she was given one cycle of neoadjuvant chemotherapy. Following this, she had profuse bleeding. Emergency hysterectomy with pelvic lymph nodal dissection was performed. The final histopathology was reported as adenosarcoma. One pelvic lymph node showed metastatic deposit of rhabdomyosarcomatous element. In young females presenting with polypoidal mass, uterine adenosarcoma can be considered in the differential diagnosis.

Keywords: Lymph node metastasis, mullerian adenosarcoma, premenopausal female, uterine sarcomas

How to cite this article:
Patnayak R, Rukmangadha N, Architha P, Reddy GS, Lakshmi AY, Kannan T, Jena A. Metastasizing uterine mullerian adenosarcoma in a young female: Diagnostic enigma. Indian J Pathol Microbiol 2018;61:440-2

How to cite this URL:
Patnayak R, Rukmangadha N, Architha P, Reddy GS, Lakshmi AY, Kannan T, Jena A. Metastasizing uterine mullerian adenosarcoma in a young female: Diagnostic enigma. Indian J Pathol Microbiol [serial online] 2018 [cited 2019 Dec 7];61:440-2. Available from: http://www.ijpmonline.org/text.asp?2018/61/3/440/236590





   Introduction Top


Uterine adenosarcomas are rarely diagnosed. It is a biphasic tumor with both epithelial and mesenchymal component. Epithelial component is benign in nature whereas the mesenchymal component is malignant. It is encountered mostly in postmenopausal women. Metastasis is rare. Its prognosis is said to be favorable.[1] The present case is unusual as it is a case of metastasizing uterine adenosarcoma in a young female.


   Case Report Top


A 20-year-old female presented with history of per vaginal bleeding for 1 month. The bleeding was heavy and associated with clots. She had occasional abdominal pain. She attained menarche at the age of 10 years and her menstrual period was irregular in nature. At presentation, she had pallor and loss of weight. Examination of the abdomen revealed a mass of 24-week size with restricted mobility. Per vaginal examination revealed a fungating mass protruding through the cervical os. There was bleeding and foul smelling discharge.

Ultrasonography and magnetic resonance imaging showed a large intrauterine mass [Figure 1]. Biopsy of the mass done at an outside hospital was reported as rhabdomyosarcoma. Hence, she was given one cycle of neoadjuvant chemotherapy. Following this, she had profuse bleeding. Emergency hysterectomy with pelvic lymph nodal dissection was performed. Both ovaries were preserved. The gross specimen showed a 20 cm × 10 cm growth protruding from the cervix. The growth was in multiple papillary masses, and the cut section was fleshy [Figure 2].
Figure 1: (a) T2-weighted sagittal: Altered signal intensity lesion heterogeneously hyperintense on T2 is noted in uterine cavity (arrow) extending to vagina measuring 16 cm × 9 cm. (b) T2-weighted coronal: Altered signal intensity lesion heterogeneously hyperintense on T2 is noted extending from uterus to vagina (arrow) bladder is displaced anteriorly

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Figure 2: (a) Protruding polypoidal growth. (b) Cut section of the growth is showing fleshy and hemorrhagic areas

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The histopathology of the polypoidal mass showed flattened epithelial lining. There were hypocellular and hypercellular areas, benign glands, and cartilaginous areas. There was condensation of stroma around the glands resulting in the formation of cambium layer. The sarcomatous area constituted more than 25% of the tumor. There were increased mitotic activity, 4–5/10 high-power field, focally noted in the stromal component. focally [Figure 3]. One pelvic lymph node showed metastatic deposit of rhabdomyosarcomatous component [Figure 4]. The final histopathology was reported as adenosarcoma with lymph nodal metastasis.
Figure 3: (a) Hypercellular area (cambium layer) beneath the flattened epithelium (H and E, ×40). (b) Stromal condensation around glands and surrounding hypocellular areas (H and E, ×40). (c) Heterologous cartilagenous elements (H and E, ×400). (d) Stromal condensation around benign looking glands (H and E, ×200)

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Figure 4: (a) Lymph node with sarcomatous metastasis (H and E, ×100). (b) Metastatic rhabdomyosarcomatous elements with polygonal neoplastic cells (H and E, ×400)

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The patient had an uneventful postoperative period and is under follow-up for the past 1½ years.


   Discussion Top


Mullerian adenosarcomas are uncommon-mixed mesenchymal and epithelial neoplasms. It was first reported by Clement and Scully.[2] They occur most commonly in the uterine corpus. Other locations of mullerian adenosarcoma in female genital tract include cervix and ovary. Vagina and fallopian tube are rarely involved.[3]

Adenosarcoma is encountered in women of all age groups. The median age is the postmenopausal group, i.e. 50–59 years, with a wide age range of 15–90 years. Extrauterine adenosarcoma is commonly seen in younger females and is more aggressive than its uterine counterpart.[3] Patients most commonly present with abnormal vaginal bleeding. Other common signs and symptoms include vaginal discharge, pain, nonspecific urinary symptoms, a palpable pelvic mass, and a tumor protruding from the cervix.[4]

Grossly, most uterine cases have an exophytic polypoid appearance. Sometimes, multiple papillary masses are seen.[2] The present case had multiple papillary masses.

Adenosarcomas are uncommon-mixed mesenchymal and epithelial neoplasms. The epithelial component is typically benign, and the mesenchymal component usually resembles that of low-grade endometrial stromal tumors.[5],[6] There is typically increased stromal cellularity around the epithelial elements, resulting in the formation of a cambium layer. The stroma is usually hormone receptor and CD10 positive.[6] Few cases show the presence of heterologous stromal elements or sex cord-like differentiation.[2] Heterologous sarcoma is seen in one-fourth of adenosarcomas with stromal overgrowth. Among heterologous sarcomama, rhabdomyosarcoma is common.[7] According to the World Health Organization definition, in adenosarcoma, the stromal mitotic activity should be 2 or more per 10 high-power fields.[6] In the present case, at places, there were increased mitotic activity, 4–5/10 high-power field. This case also had cartilaginous areas, and the lymph node showed metastatic rhabdomyosarcomatous deposits.

The stromal component is responsible for the clinical behavior. Thus, the differential diagnosis spectrum includes low-grade endometrial stromal tumors, adenofibroma, carcinosarcoma, and in children, botryoid rhabdomyosarcoma.[5],[6],[8] There are few similarities between adenosarcoma and botryoid rhabdomyosarcoma. Both the entities show polypoid growth and stromal condensation beneath epithelium. Probably because of these factors, the present case was diagnosed as an embryonal rhabdomyosarcoma in an outside center. However, in botryoid rhabdomyosarcoma, more typically conspicuous myxoid stroma is present, and it shows a sprinkling of small cellular aggregates of dark blue, primitive, and mitotically active cells in a paucicellular background. In contrast to adenosarcoma, rhabdomyosarcoma does not exhibit phyllodes-like growth or intraglandular stromal papillae. The entity adenosarcomas with stromal overgrowth frequently contain rhabdomyoblastic foci. However, whether there exist significant clinical differences between such tumors and pure rhabdomyosarcoma is not clearly defined. It is said that some rhabdomyosarcomas might represent adenosarcomas with complete heterologous stromal overgrowth.

Lymph nodal metastasis is rare. Occasional patients with sarcomatous overgrowth may have pelvic lymph node metastases.[9]

Choi et al. have reported a case of uterine müllerian adenosarcoma with sarcomatous overgrowth and lung metastasis. Uterine müllerian adenosarcoma with sarcomatous overgrowth is a rare variant of uterine adenosarcomas. Here, sarcomatous portion constitutes more than 25% of the tumor. It usually presents as a protruding cervical polyp. It is very rare in premenopausal women. This tumor is a highly aggressive in nature.[1] The present case had many similar features as described by Choi.[1]

There is no defined best approach for management of uterine adenosarcomas. All the modalities such as surgery, chemotherapy, and radiotherapy have all been used alone or in combination.[10]


   Conclusion Top


In young females presenting with polypoidal mass, uterine adenosarcoma can be considered in the differential diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Choi YJ, Jung MH, Park YK, Lee BY, Huh CY. Uterine müllerian adenosarcoma with sarcomatous overgrowth and lung metastasis in a 25-year-old woman. Eur J Gynaecol Oncol 2011;32:91-4.  Back to cited text no. 1
    
2.
Clement PB, Scully RE. Müllerian adenosarcoma of the uterus. A clinicopathologic analysis of ten cases of a distinctive type of müllerian mixed tumor. Cancer 1974;34:1138-49.  Back to cited text no. 2
    
3.
McCluggage WG. Mullerian adenosarcoma of the female genital tract. Adv Anat Pathol 2010;17:122-9.  Back to cited text no. 3
    
4.
Verschraegen CF, Vasuratna A, Edwards C, Freedman R, Kudelka AP, Tornos C, et al. Clinicopathologic analysis of mullerian adenosarcoma: The M.D. Anderson Cancer Center experience. Oncol Rep 1998;5:939-44.  Back to cited text no. 4
    
5.
Zaloudek CJ, Norris HJ. Adenofibroma and adenosarcoma of the uterus: A clinicopathologic study of 35 cases. Cancer 1981;48:354-66.  Back to cited text no. 5
    
6.
Soslow RA, Ali A, Oliva E. Mullerian adenosarcomas: An immunophenotypic analysis of 35 cases. Am J Surg Pathol 2008;32:1013-21.  Back to cited text no. 6
    
7.
Clement PB. Müllerian adenosarcomas of the uterus with sarcomatous overgrowth. A clinicopathological analysis of 10 cases. Am J Surg Pathol 1989;13:28-38.  Back to cited text no. 7
    
8.
D'Angelo E, Prat J. Pathology of mixed müllerian tumours. Best Pract Res Clin Obstet Gynaecol 2011;25:705-18.  Back to cited text no. 8
    
9.
Seidman JD, Wasserman CS, Aye LM, MacKoul PJ, O'Leary TJ. Cluster of uterine mullerian adenosarcoma in the Washington, DC metropolitan area with high incidence of sarcomatous overgrowth. Am J Surg Pathol 1999;23:809-14.  Back to cited text no. 9
    
10.
Tinar S, Sehirali S, Inal MM, Yildrim Y, Celik E, Yigit S, et al. Adenosarcoma of the uterus: A case report. MedGenMed 2004;6:51.  Back to cited text no. 10
    

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Correspondence Address:
Rashmi Patnayak
Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati - 517 507, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_1_18

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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