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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 1  |  Issue : 2  |  Page : 40-42

Vaginal primary amelanotic melanoma with survival beyond expectation: A rare case report


1 Department of Gynaecological Oncology, Acharya Harihar Regional Cancer Centre, Cuttack, Odisha, India
2 Department of Pathology, Acharya Harihar Regional Cancer Centre, Cuttack, Odisha, India

Date of Web Publication19-Jan-2018

Correspondence Address:
Bhagyalaxmi Nayak
Department of Gynaecological Oncology, Acharya Harihar Regional Cancer Centre, Cuttack - 753 007, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/oji.oji_18_17

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  Abstract 

Vaginal malignancies are uncommon, constituting 1%–2% of all the female genital tract malignancies. Even rare are melanomas of the vagina with an annual incidence of 0.026/100,000 women worldwide, and amelanotic variant constitutes only 2% of all the vaginal melanomas. Here, we report a case of primary vaginal amelanotic melanoma in a 70-year-old woman, initially treated with wide local excision followed by adjuvant chemotherapy. After six and half years of follow-up, the patient developed a recurrence in the left inguinal nodal region with a suspicious pulmonary nodule and is now under treatment. Amelanotic melanoma has varied clinical presentation and microscopic appearance with the absence of characteristic pigmentation, leading to a diagnostic delay and thus carries a poor prognosis. Therefore, it is prudent to have a high index of suspicion for these rare tumors. Hence, we report this case of primary vaginal melanoma, amelanotic variant with a surprisingly long survival of nearly 7 years for information and pertinent discussion.

Keywords: Amelanotic melanoma, primary, survival, vagina


How to cite this article:
Kulkarni RV, Nayak B, Giri SK, Samantray S. Vaginal primary amelanotic melanoma with survival beyond expectation: A rare case report. Oncol J India 2017;1:40-2

How to cite this URL:
Kulkarni RV, Nayak B, Giri SK, Samantray S. Vaginal primary amelanotic melanoma with survival beyond expectation: A rare case report. Oncol J India [serial online] 2017 [cited 2021 Dec 5];1:40-2. Available from: https://www.ojionline.org/text.asp?2017/1/2/40/223682




  Introduction Top


Vaginal malignancies are rare, representing 1%–2% of all female genital tract malignancies.[1] Even rare are melanomas of the vagina which constitute <3% of all the vaginal cancers and <1% of all malignant melanomas and 2%–5% of female genital tract melanomas.[2],[3] The annual incidence of vaginal primary malignant melanoma (VPMM) is 0.026/100,000 women worldwide.[3] About 500 of VPMM have been reported in modern literature.[4] However, primary vaginal melanoma of amelanotic variant is extremely rare and constitutes only 2% of all vaginal melanomas.[5] Here, we report a case of primary vaginal melanoma, amelanotic variant with a surprisingly long survival of 7 years.


  Case Report Top


A 70-year-old postmenopausal woman presented with a swelling over the left inguinal region close to mons pubis for 2 months. She had a history of postmenopausal bleeding about 7 years ago, which was evaluated and she was found to have a 3 cm polypoidal growth at the suburethral region over the anterior vaginal wall. She underwent wide local excision for the same; histopathology was reported as poorly differentiated carcinoma. Immunohistochemistry (IHC) was negative for CK, leukocyte common antigen (LCA), CK–5/6 but positive for HMB-45 in atypical cells. Hence, a diagnosis of amelanotic melanoma was arrived at. Following this, a metastatic workup was done and none found. Due to high grade of the disease, she received four cycles of adjuvant chemotherapy with vinblastine and carboplatin. She was disease free for a period of six and half years following which she developed a swelling near the left mons and evaluated for the same elsewhere. Fine needle aspiration cytology (FNAC) from the swelling found to be metastasis, and she underwent left inguinal node dissection. Two out of the six resected nodes were positive for metastatic melanoma. She received oral temozolomide for a period of 5 months. A month later, contrast-enhanced computed tomography (CECT) of the abdomen and pelvis showed an enlarged lymph node (30 mm × 28 mm) in the left inguinal region [Figure 1]a; FNAC of the lymph node proved to be metastatic amelanotic melanoma. Following this, the patient reported to our hospital. On examination, a lymph node of size 4 cm × 4 cm found over the left inguinal node without any fixity, skin infiltration, or ulceration. We did a metastatic workup including CECT thorax in addition to CECT abdomen and pelvis and found no other lesions other than a small nodule (15 mm × 14 mm × 16 mm) in the posterior basal segment of the left lung [Figure 1]b. After a multidisciplinary team discussion, lymphadenectomy was performed, showing amelanotic melanoma recurrence on histopathological examination [Figure 2]a and [Figure 2]b. IHC of the lymph node dissection specimen showed positivity of HMB-45 and S100 in amelanotic tumor cells and negativity of CK and LCA confirming the diagnosis of amelanotic melanoma [Figure 3]a and [Figure 3]b. The patient is now under the care of medical oncology department for chemotherapy.
Figure 1: (a) Contrast-enhanced computed tomography scan of the pelvis showing enlarged left inguinal lymph node, and (b) contrast-enhanced computed tomography scan of the thorax showing a pulmonary nodule 15 mm × 16 mm at the posterior basal segment of the left lung

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Figure 2: Photomicrography showing (a) lymphoid tissue with metastatic deposits (H and E, ×100), and (b) nests of tumor tissue with lymphocytes and areas of necrosis (H and E, ×400)

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Figure 3: Photomicrography of immunohistochemistry from the dissected lymph node specimen showing diffuse strong positivity of (a) S100 – ×400, and (b) HMB-45 – ×400, in amelanotic tumor cells

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  Discussion Top


Three percent of healthy women harbor melanocytes in the basal layer of the vaginal epithelium as embryological remnants of neural crest cells, and VPMM is thought to arise from these aberrantly located melanocytes.[4] There are no identifiable risk or etiological factors associated with these tumors, and the mechanism of pathogenesis is still unknown.[3]

Vaginal melanomas usually present in the sixth and seventh decades of life, and majority of the cases arise from the lower one-third of the anterior vaginal wall as in the present case. It may be single or multiple, pigmented or nonpigmented, and also, polypoidal or ulcerated. Frequently, they present with vaginal bleeding (80%) followed by vaginal discharge, feeling a mass in the vagina, and pain.[6] Most of these tumors are pigmented (brown or black) and only about 10%–23% are amelanotic, making our case further exceptional.[2]

Amelanotic melanoma has a varied clinical and microscopic appearance. It can be defined either clinically as melanomas devoid of pigment on visual inspection before biopsy or absence of melanin pigment in melanoma cells on routine H and E-stained sections. Previous data showed an association of older age, head/neck site, and sun-damaged skin with amelanotic melanoma. It can present in nodular or ulcerated forms, nodular being predominant.[7] It is difficult to differentiate amelanotic melanoma from other epithelial and nonepithelial malignancies and is initially misdiagnosed as carcinomas or sarcomas because of the minimal number of melanin granules. Therefore, HMB-45 and S-100 protein positivity on IHC study confirm the diagnosis of amelanotic melanoma.[8] Amelanotic melanomas have poorer melanoma-specific and overall survival possibly due to delayed diagnosis of this subset of melanomas in view of their inconspicuous nature.

Controversy exists in the optimal treatment for these cases. Surgery is the preferred option for the resectable cases. Wide local excision is the common surgical approach for early stage tumors. Adjuvant radiotherapy may be considered in early stage disease. In locally advanced cases, a radical surgery is done with lymphadenectomy including neoadjuvant or adjuvant chemotherapy or radiotherapy.[9] Because of low rate of lymph node metastasis, lymph node dissection is not recommended for VPMM and should be reserved for patients with clinically or radiologically enlarged nodes. Radiotherapy may be used preoperatively to facilitate a more conservative surgery and can be used in adjuvant treatment for incomplete surgery or with pelvic metastasis. In advanced stages, chemotherapeutic options include dacarbazine, temozolomide, paclitaxel, nitrosourea, imatinib, nimustine, vincristine, and cisplatin/carboplatin. Our patient had received a combination of vinblastine and carboplatin as adjuvant treatment and later temozolomide during recurrence considering patient's age, general condition, and her preference owing to its ease of administration and limited side effect profile.[10] There are reports of increased response rate with the use of biochemotherapy (combination of chemotherapy and immunotherapy) in advanced VPMM.[3]

The prognosis is poor including early local recurrence, lymph node involvement, and distant metastasis. The tumor size more than 3 cm is the most important prognostic factor. The stage of the disease and histopathological variant also play a role in prognosis. Most of the vaginal melanomas are aggressive in nature and tend to be diagnosed at late stages with a 5-year survival rate of 0%–25% and this dwindles down to 5% in 3 years with lymph node involvement.[4] Considering these statistics and the fact that our patient had amelanotic variant with poorer prognosis including lymph node involvement, it is safe to say that she has had an impressive survival.

The present case is noteworthy with a survival of about 7 years and demands further research in the area of its early detection and optimal management for better outcomes.

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.

Acknowledgment

We profusely thank Dr. Manas R Baisakh MD (Path); PDCC (Oncopath), Sr. Consultant Histopathologist, Apollo Hospitals, Bhubaneswar, for his immense help and support in immunohistochemical analysis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Berek JS, Hacker NF. Vaginal cancer. Berek & Hacker's Gynaecologic Oncology. 6th ed. Ch. 14. Philadelphia: Wolters Kluwer; 2015. p. 608.  Back to cited text no. 1
    
2.
Chaudhuri S, Das D, Chowdhury S, Gupta AD. Primary malignant melanoma of the vagina: A case report and review of literature. South Asian J Cancer 2013;2:4.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Androutsopoulos G, Terzakis E, Ioannidou G, Tsamandas A, Decavalas G. Vaginal primary malignant melanoma: A rare and aggressive tumor. Case Rep Obstet Gynecol 2013;2013:137908.  Back to cited text no. 3
    
4.
Kalampokas E, Kalampokas T, Damaskos C. Primary vaginal melanoma, A rare and aggressive entity. A case report and review of the literature. In Vivo 2017;31:133-9.  Back to cited text no. 4
    
5.
Satyanarayan JN, Nangal J, Kapoor A, Sharma N. Vaginal amelanotic nodular malignant melanoma in A middle-aged female: A rare case report and review of literature. J Obstet Gynaecol India 2015;65:199-201.  Back to cited text no. 5
    
6.
Terzakis E, Androutsopoulos G, Adonakis G, Zygouris D, Grigoriadis C, Decavalas G, et al. Vaginal primary malignant melanoma: Report of four cases and review of the literature. Eur J Gynaecol Oncol 2011;32:122-4.  Back to cited text no. 6
    
7.
McClain SE, Mayo KB, Shada AL, Smolkin ME, Patterson JW, Slingluff CL Jr., et al. Amelanotic melanomas presenting as red skin lesions: A diagnostic challenge with potentially lethal consequences. Int J Dermatol 2012;51:420-6.  Back to cited text no. 7
    
8.
Oguri H, Izumiya C, Maeda N, Fukaya T, Moriki T. A primary amelanotic melanoma of the vagina, diagnosed by immunohistochemical staining with HMB-45, which recurred as a pigmented melanoma. J Clin Pathol 2004;57:986-8.  Back to cited text no. 8
    
9.
Xia L, Han D, Yang W, Li J, Chuang L, Wu X, et al. Primary malignant melanoma of the vagina: A retrospective clinicopathologic study of 44 cases. Int J Gynecol Cancer 2014;24:149-55.  Back to cited text no. 9
    
10.
Jelić S, Babović N, Stamatović L, Kreacić M, Matković S, Popov I, et al. Vinblastin-carboplatin for metastatic cutaneous melanoma as first-line chemotherapy and in dacarbazine failures: A single-center study. Med Oncol 2001;18:189-95.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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