Ovarian metastasis of malignant melanoma: The first pediatric case

Ovarian metastasis of malignant melanoma: The first pediatric case

J Ped Surg Case Reports 2 (2014) 473e475 Contents lists available at ScienceDirect Journal of Pediatric Surgery CASE REPORTS journal homepage: www.j...

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J Ped Surg Case Reports 2 (2014) 473e475

Contents lists available at ScienceDirect

Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com

Ovarian metastasis of malignant melanoma: The first pediatric caseq Yuko Araki a, *, Hide Kaneda a, Kohei Oashi b, Sathoshi Okada c, Arata Tsutsumida b a

Department of Pediatric Surgical Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan Department of Dermatologic Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan c Department of Gynecology, Tokyo Metropolitan Bokutoh Hospital, 4-23-15 Kotobashi, Sumida-ku, Tokyo 130-8575, Japan b

a r t i c l e i n f o

a b s t r a c t

Article history: Received 24 July 2014 Received in revised form 16 September 2014 Accepted 17 September 2014

We report a case of an 8-year-old girl with metastasis of malignant melanoma (MM) to the ovary. She was initially diagnosed with cutaneous MM on the left buttock for which she underwent wide local excision, left inguinal/pelvic lymph node dissection, and subcutaneous injection of interferon beta. In spite of the treatment, she developed dissemination of MM to the liver, the bone, and the right ovary. All the lesions responded well to systemic chemotherapy (intravenous dacarbazine), except for the right ovarian tumor. She underwent an elective right salpingo-oophorectomy to avoid torsion or rupture of the tumor. However, she developed metastases to the contralateral ovary with peritoneal dissemination in 4 months. She received home palliative care and died at home 14 months after the last surgery. Ovarian metastasis of MM is a rare form of dissemination, and only 15 adult cases have ever been reported. Our patient is the first pediatric case. Since there is no standard of surgical indication for metastatic MM to the ovary, palliative resection can be an option for improving quality of life of a patient with this rare condition. Ó 2014 The Authors. Published by Elsevier Inc. All rights reserved.

Key words: Malignant melanoma Ovarian tumor Metastasis

Metastasis of malignant melanoma to the ovary is a rare form of dissemination, and no pediatric case has ever been reported. We present a case of an eight-year-old girl, the first case report of this condition in pediatric population.

1. Case report The patient is an 8-year-old girl who was diagnosed with malignant melanoma (MM) on the left buttock. At the age of 3, she underwent excision of congenital melanocytic nevi on the left buttock. When she was 6 years old, a black-colored skin lesion was noticed in the same area which rapidly increased in size over 6 months. Incisional biopsy of the lesion confirmed pathological diagnosis of MM. She underwent wide local excision of the lesion plus sentinel lymph node (LN) biopsy which was positive (pT4bN2bM0, stage IIIB; Clark’s level V, ulceration (þ)). She was referred to our institution for LN dissection of the left inguinal area

q This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/3.0/). * Corresponding author. Tel.: þ81 3 3542 2511; fax: þ81 3 3545 3567. E-mail address: [email protected] (Y. Araki).

and the pelvis. Postoperatively, she received subcutaneous injection of interferon beta (3 million units per day, total 24 doses). However, she developed dissemination of MM to the liver, bone (the spine (Th12) and the ilium), and the right ovary. Her spine was with compression fracture. She received radiation therapy to the spine lesion (45 Gy/18 fractions) and systemic chemotherapy (intravenous dacarbazine, 1000 mg/m2/day, given every 2 weeks  4). Although the liver and bone lesions responded well to treatments, her right ovary enlarged from 2 cm to 6 cm within a month (Fig. 1). Serum tumor markers were normal except elevated LDH. To avoid an acute abdomen from ovarian torsion or rupture, she underwent an elective right salpingo-oophorectomy. Total 1200 ml of bloody ascites was evacuated at laparotomy, and right salpingo-oophorectomy was completed. Post-operative course was uncomplicated and she was discharged from the hospital on post-operative day 8. Pathologic examination confirmed diagnosis of metastatic melanoma of the ovary; macroscopic examination revealed an elastic-soft nodule (2.5  1.5  0.8 cm) inside of the thinned ovarian capsule. Immunohistochemical stains were positive for HMB-45 and antiMelan-A (Fig. 2). In spite of additional 13 doses (total 17 doses) of intravenous dacarbazine, her CT scan taken at 4 months from surgery showed enlarged left ovary accompanied by multiple peritoneal

2213-5766/$ e see front matter Ó 2014 The Authors. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.epsc.2014.09.010

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Y. Araki et al. / J Ped Surg Case Reports 2 (2014) 473e475

Fig. 1. Preoperative CT of the right ovary, measured 6  5 cm.

disseminations and ascites. She received home palliative care and died at home 14 months after the last surgery. 2. Discussion MM of children is rare, but has been increasing. It consists 1e3% of pediatric malignancies, and only 0.4% of all malignant melanoma cases [1]. According to Surveillance, Epidemiology, and End Results (SEER) program data from 1973 through 2009, the age-adjusted incident rate of MM in children and adolescents less than 20 years of age was 6.0 per million and increased by an average of 2% per year [2]. Ovarian metastasis of MM is a rare form of disseminated MM, and only 15 adult cases have been reported (Table 1) [3e17]. The youngest patient in the previous reports was a 20-year-old [3]. Therefore, this is the youngest report of MM in the ovary. Symptoms of the ovarian metastasis vary from asymptomatic to acute abdomen (abdominal pain, abdominal distention, shock) [3e17]. Eleven out of the 15 cases were seen with sudden onset of abdominal pain [3e7,9,11,14e17], and 3 of them had rupture of the ovarian tumor prior to laparotomy [3,5,16]. Prognosis of MM with distant metastases is poor. According to Barth et al., the median survival time of the 1521 patients they

studied was 7.5 months, and the estimated five-year survival rate was 6 percent [18]. Metastasis of MM to the ovary has unpredictable clinical and biological behavior and poor prognosis due to the aggressiveness of the tumor [16]. Ten out of the 15 cases were reported to be dead [3,6,8,10,12e17], and seven died within a year after surgery to the ovary [6,8,10,12,13,15,16]. Because of the rarity of this form of metastasis, there is no standard surgical indication for ovarian MM. When reviewing the 15 cases in Table 1, there could be a chance of improved survival with oophorectomy in late recurrent cases; among eight cases which developed ovarian metastases five years or beyond from the initial diagnosis of MM, five cases (62.5%) survived one year or beyond after oophorectomy [7,9,10,14,17]. The National Comprehensive Cancer Network (NCCN) guideline offers palliative resection and/or radiation as an option for symptomatic patients with disseminated MM [19]. Although our patient was asymptomatic, we performed an elective right oophorectomy for several reasons. First, as the ovary was rapidly enlarging, we wanted to avoid having to perform an emergency laparotomy to remove a ruptured ovary. Secondly, the right oophorectomy would yield several benefits: 1) after removal of the large tumor, systemic chemotherapy would likely be more effective; 2) removal of the large tumor would also better facilitate supportive care as the risks of an emergency operation would be reduced. Another reason for selection of a right oophorectomy over a bilateral was the patient’s young age. Although gynecologists more commonly perform bilateral oophorectomy on similar adult patients, we felt that in this situation a more conservative approach was warranted. The parents expressed a strong desire to preserve one ovary. Although four months later, the patient developed left (contralateral) ovarian metastasis plus peritoneal dissemination, we felt our original approach was still warranted. 3. Conclusion We reported on an 8-year-old girl with ovarian metastasis of MM. Since no standard therapy is established on this form of metastasis and the ovarian tumor can enlarge to induce lifethreatening acute abdomen, palliative resection (salpingo-oophorectomy) can be an option for improving the QOL of the affected patient. Indication of surgery should be decided weighing the risks

Fig. 2. Histopathology of the right ovarian tumor showing metastasis of malignant melanoma. (A) Hematoxylin and eosin stain (100). (B) Anti-HMB45 stain (400). (C) Anti-Melan-A stain (400).

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Table 1 Published cases of metastasis of malignant melanoma to the ovary. No.

Age (years)

Laterality of the affected ovary

Clinical symptoms

Surgery

Site of primary lesion

Time from initial diagnosis to ovarian metastasis

Outcome (time from surgery)

Author (year)

1 2

20 40

Rt Lt

Abd pain Abd pain

SO (Rt) SO (Bil), H

9 Mo (1) 7 Mo (2) 1 Mo

Died (NA) Alive (NA)

Silveira E (1977) Agnello (1981)

3

42

Rt

Abd pain, shock

2 Yr

NA

Nanu VK (1985)

4

42

Bil

Abd pain, malaise

Skin (right heel)

16 Mo

Died (2 Mo)

Stjernholm J (1991)

5

46

Bil

Skin (vulva)

10 Yr

Alive (1 Yr)

Meier HM (1995)

6

35

Bil

Abd pain, urinary incontinence Abd mass

SO (Rt); 2 L of blood evacuated SO (Bil), H, OM and appendectomy SO (Bil), H, OM, and appendectomy SO (Bil)

Skin (left breast) (1) Skin (left paraumbilical zone); (2) skin (left breast) Skin (right calf)

8 yr

Died (5 Mo)

Bilgin T (2000)

7

47

Rt

Abd pain

SO (Rt)

Skin (temporal scalp) Right eye (uvea)

14 Yr

Alive (14 Mo)

8

58

Rt

Abd mass

Skin (left antecubital fossa)

10 Yr

Died (1 Yr)

9 10

48 43

Bil Lt

Abd pain NA

NA Died (8 Mo)

Al-Shehabi Z (2006) Boutis A (2008)

39

Lt

8 Mo

Died (7 Mo)

Abe Y (2009)

12

35

Bil

Skin (upper back)

5 Yr

Died (15 Mo)

Fenzl V (2011)

13

45

Lt

Abd fullness, appetite loss Abd pain, fever, chills, fatigue, appetite loss and nausea Abd mass

Skin (left thigh) Skin (left antecubital region) Skin (left thigh)

2 Yr 9 Yr

11

SO (Bil), H, OM, and right pelvic lymphadenectomy SO (Bil), H US-guided aspiration of ascites SO (Lt), OM and peritoneal Bx SO (Bil), OM

Santeusanio G (2000) Oliver R (2005)

SO (Lt)

3 Yr

Died (11 Mo)

Sbitti (2011)

14

35

Rt

Abd pain, shock

SO (Rt)

7 Yr

Died (3 Mo)

Habek D (2012)

15

48

Lt

Abd pain

5 Yr

Died (4 Yr)

Berisavac M (2013)

16

8

Bil (metachronous)

Asymptomatic

SO (Bil), H, OM and pelvic lymphadenectomy SO (Rt)

Skin (sole of the right foot) Skin (interscapular region) Skin (right arm)

Skin (left buttock)

8 Mo

Died (14 Mo)

Araki Y (2014)

NA, not available; SO, salpingo-oophorectomy; H, hysterectomy; OM, omentectomy.

and benefits of surgery, with limited life expectancy of the patient in mind.

[10]

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