Kimura, Ryoko

Shiomi, Tatsushi

Suzuki, Shuji

Yamamoto, Osamu

Yanagihara, Shigeto

Yoshida, Yuichi

Tottori University Research Result Repository

183Yonago Acta medica 2016;59:183–187 Patient ReportCorresponding author: Shuji Suzuki, MD, PhDdrshuji@yahoo.co.jpReceived 2016 March 8Accepted 2016 April 6Abbreviation: CT, computed tomographyMelanoma of the Umbilicus: A Patient Report, Precaution in Operative Strategy, and the First Histopathological Review of Published CasesShuji Suzuki,* Yuichi Yoshida,* Tatsushi Shiomi,† Shigeto Yanagihara,* Ryoko Kimura* and Osamu Yamamoto**Division of Dermatology, Department of Medicine of Sensory and Motor Organs, School of Medicine, Tottori University Faculty of Medicine, Yonago 683-8503, Japan and †Division of Organ Pathology, Department of Pathology, School of Medicine, Tottori University Faculty of Medicine, Yonago 683-8503, JapanABSTRACTUmbilical melanoma is extremely rare. Among the past English reports on umbilical melanoma, there are only 8 reports in which histopathology was described in detail, and there has been no report with a review of the histo-pathology of previously reported cases.  We experienced a case of umbilical melanoma and reviewed previously reported cases including our case. Because of the anatomical location, it is difficult to become aware of the umbilical melanoma unless there are some concomitant symptoms such as discharge or swelling. Even with these symptoms, patients tend to postpone a hospital visit for unknown reasons, resulting in increased risk of tumor growth and metastasis. When performing resection of umbilical melanoma, a portion of the peritoneum should also be removed. Sentinel lymph nodes can be axilla or inguinal lymph nodes. There is a possibility of metastasis to the preoperative abdominal cavity or to nearby skin through hematog-enous spread. Preoperative evaluation of tumor spread and postoperative observation are important for umbili-cal melanoma in order to detect recurrence or metastasis because of its unique anatomical location.Key words    abdominal hernia; peritoneum; prognosis; sentinel lymph node; umbilical melanomaPrimary melanoma arising in the umbilicus is extremely rare. Its anatomical location makes it difficult for the pa-tients to realize the abnormality and challenges surgeons in the treatment strategy. Since Cullen reported the first 3 cases in 1916, 24 cases have been reported in the English literature; however, only 8 cases were reported with sufficient histopathological description.1–13 There has been no review of the histopathology of reported cases. We present a case of melanoma that arose in the um-bilicus. We also discuss 7 reports of 8 cases of primary umbilical melanoma with histopathology, locations of sentinel lymph nodes, metastasis, and operative treat-ment.PATIENT REPORTAn 83-year-old woman presented to our hospital with an umbilical skin tumor. She first noticed it with exudates and pruritus 2 weeks earlier, saw a local physician, and was referred to us. She had a past medical history of a gastric cancer operation 26 years ago. There was a 12 × 6 mm black nodule arising in the umbilicus without an ulcer on the surface (Figs. 1a and b). No lymph node swelling was palpable on either side of the inguinal and axillary areas.  Biopsy of the nodule revealed a solid proliferation of atypical melanocytes of epithelioid and spindle shapes with tumor thickness exceeding 2 mm. Numerous mi-toses were seen and there was no maturation towards the deeper side. Immunostainings against S-100 protein, HMB-45, and Melan-A were all positive. Computed tomography (CT) revealed no metastasis or lymph node swelling. At that time, we diagnosed the nodule as mel-anoma, T3a (or 4a) N0M0, stage IIA (or IIB). Lympho-scintigraphy with intradermic injections of 99mTc near the nodule showed accumulation of gamma-emitting lymph nodes on both sides of the inguinal areas (Fig. 1c).   Excision with a 2.0 cm margin and sentinel lymph node biopsy of both sides of the inguinal areas were performed after injecting blue dye. The excision line in-cluded part of the previous operation scar (Fig. 1d). The peritoneum under the navel was also included within the excision (Fig. 1e). The fascia of each layer and the skin was sutured without obvious tension that might cause the sutures to break at this point. On the next day after surgery, there was a noticeable swelling of the abdominal wall, and CT of the abdomen revealed an abdominal hernia arising from the umbilical area (Fig. 1f). An emergent radical operation for the her-nia using a silicon mesh sheet was performed.  Histopathology of the tumor showed atypical me-lanocytic proliferation in the dermis invading into the subcutaneous fat layer without maturation (Fig. 1g). 184S. Suzuki et al.Fig. 1. Clinical and operative view, image study, and histopathology.a: A pigmented nodule of 2 × 1 cm in the umbilicus. b: The umbilicus was filled with the nodule. c: Lymphoscintigraphy with intradermic injections of 99mTc near the nodule. Accumulation of gamma-emitting lymph nodes on both sides of the inguinal area was visible, being more prominent on the right side. d: Incision line included the lower part of the previous operation scar of gastrectomy. e: A portion of the peritoneum was removed and a part of the jejunum is exposed (red arrow). f: Computed tomography on the first day after tumor resection. A part of the jejunum herniated under the skin (red ellipse). g: Atypical melanocyte proliferation in the dermis close to the subcutaneous fat layer (hematoxylin and eosin). Bar = 1 mm. h: Some of these cells were in mitosis and no maturation was observed. (hematoxylin and eosin). Bar = 20 μm.185Umbilical melanoma histopathologyTable 1. Umbilical melanomasAge (years old) SexTime between onset/first visitSize Clark’slevelBreslow tumor thicknessDeath/ AliveMelanoma subtypeLocation of SLN MetastasisPerito-neum excisionColonna 1999458 f Not described 4 cm VNot described (> 4mm, apparently)Death, 10 months after resectionNodular Not examined2 satellite skin lesions+Colonna 1999430 m 18 mo. > 10 cm (size of fist) VNot described (> 4mm, apparently)Death, 7 monthsafter initial visitNot describedNot examinedMultiple abdominal and pelvic metastases(Surgeryrefused)Meine 2003569 f 2 mo. 1.4 × 2.4 cm III 1 mm Alive 3 ySuperficial spreadingNot examined ––/+ (Second surgery)Campos-Munoz 2007634 f 3 mo. 1.2 cm III 1.06 mm Alive 18 moSuperficial spreadingLeft axilla without metastasis– +Mangas 2008763 m Unknown timeNot described III 0.8 mmAlive 6 moSuperficial spreadingNot examined –Not mentionedCecchi 2009872 f 4 y 1.5 × 3.0 cm IV 4.3 mm Alive 1 ySuperficial spreading + nodularBoth inguinal without metastasis– +Zaccagna 2010960 f Not described1.8 × 2.3 cm IV 2.8 mmAlive 86 moNot describedLeft axilla without metastasis– +Song 20131362 m 4 y 2 × 1 cm IV 3 mm Alive 36 moNot describedNot examined – +Presentcase83 f 14 days 1.2 × 0.6 cm IV 11 mmAlive 15 mo NodularBoth uinal without metastasis– +CT, computed tomography; f, female; m, male; mo, month(s); MRI, magnetic resonance imaging; PET, positron emission tomography; SLN, sentinel lymph node; US, ultrasonography; y, year(s).Frequent mitoses were observed. Clark’s level and Breslow’s tumor thickness were IV and 11 mm, respec-tively (Figs. 1g and h). No melanoma cells were seen in sentinel lymph nodes. The case was diagnosed as mela-noma, pT3aN0M0, stage IIA. There have been no signs of recurrence or metastasis of the tumor or of abdominal hernia for 15 months after surgery.DISCUSSIONClark et al. described the histopathological level of cuta-neous melanoma cell invasion and prognosis in 1969.14 A review of the histopathology of umbilical melanoma in relation to prognosis was not done until Colonna et al. reported 2 cases of umbilical melanoma in 1999.4 Many case reports of umbilical melanoma lack histopatho-186S. Suzuki et al.logically sufficient evaluation, making it questionable whether the lesions were melanoma or primary lesions. Taking these factors into account, 9 cases including the present case are considered to be histopathologically determined primary umbilical melanoma (Table 1). The age of those patients ranged from 30 to 83 years with an average age of 59 years. The patients included 2 Cauca-sians, 2 Asians, and 5 patients without ethnical descrip-tion. Six patients were female. The present case had the largest described tumor thickness of 11 mm among the 9 cases, but the patient became aware of the tumor only 14 days before the ini-tial visit to a local physician, suggesting that the tumor had existed for a considerably long time. Her abdominal skin sagged and the umbilicus area was not easily visi-ble. She went to hot spas with her daughter about once a month for many years but she first noticed the umbilical nodule when she experienced itching and bleeding after the scratching. There was also a delay in medical care in many of the previously reported cases. Among 5 cases for which there was description about the time between the patient’s realization of the lesion and the first med-ical visit, there were delays of 18 months or more in 3 cases and 2 to 3 months in 2 cases (Table 1). There was a tendency not to visit a physician at an early stage if there were no concomitant symptoms such as discharge or swelling. Sagging skin caused by obesity or aging might be the main reason for not being aware of umbilical le-sions. Other patients were aware of the nodules, but they did not seek medical care for a long time for unknown reasons, and the delay resulted in growth and possible metastasis of the tumor.  The long-term prognosis of umbilical melanoma is not clear. Among the histopathologically determined cases, only 2 cases had a follow-up period of 3 years or longer, and the size and thickness of the tumor seem to be important in evaluation of prognosis. Two patients reported died within one year (10 months after resection of the tumor and 7 months after refusing treatment). Both patients had tumors of 4 cm or larger, and, al-though not specifically described, probably had Breslow tumor thickness far larger than 4 mm judging from the clinical pictures showing large nodules.4 Breslow tumor thickness of our case was also very large (11 mm) and there has been no sign of recurrence or metastasis for 15 months after the surgery. It is important to continue to follow up this patient. Four cases were described with histopathological subtypes, superficial spreading in 2 cases, nodular in one case, and both components mixed in the same lesion in one case, and the present case is the second reported case of nodular subtype (Table 1). The most unique feature of umbilical melanoma is its anatomical location. Being the embryological rem-nant of the conduit between the embryo and the placen-ta, primary melanoma in the umbilicus may metastasize to the abdominal cavity through ligaments derived from the umbilical vein, umbilical arteries, the omphalomes-enteric duct, or remnants of the urachus if present.9 The tumor may metastasize directly to the adjacent skin through lymphatic or hematogenous spread or to the sentinel lymph nodes. Two cases actually showed me-tastases: one case had metastasis to adjacent skin and the other case had metastases to multiple abdominal and pelvic organs and to internal thoracic and external iliac lymph nodes.4 Among cases in which sentinel lymph nodes were examined, it was found in axillary nodes in 2 cases and in inguinal nodes in 2 cases including the present case.6, 8, 9 Because of the possibility of metasta-ses through remnant ligaments, 6 of 7 previous reports supported resection of a part of the peritoneum below the umbilicus.4–6, 8, 9, 13 In fact, one case first underwent resection of the fascia level, but remnant melanoma was later found and a second operation was performed one year later to remove the remnant tumor and a portion of the peritoneum.5 Regarding the operative treatment approach, all operated cases followed the WHO Melanoma trial for the resection margin, but only one report described aug-mentation of the abdominal cavity with pre-peritoneal synthetic mesh.4, 15 We first performed resection of a part of the peritoneum and closed the abdominal cav-ity, which resulted in postoperative abdominal hernia. Her rectus abdominis may have been brittle due to the previous gastric cancer surgery and her advanced age. Her abdominal cavity should have been reinforced with pre-peritoneal synthetic mesh at the time of resection. Even without pre-surgical fragility of the abdominal wall, resection of the umbilical melanoma with a portion of the peritoneum should be considered with synthetic mesh augmentation. Umbilical melanoma has a greater chance of tumor growth than does skin melanoma of other areas at the time of the first presentation. Pre-surgical evaluation of tumor spread is necessary, and the resection should be deep enough to include a portion of the peritoneum be-neath the lesion.The authors declare no conflict of interest. REFERENCES  1 Cullen TS. Sarcoma of the umbilicus. In: Embryology, anato-my, and diseases of the umbilicus, together with disease of the urachus. Philadelphia: W.B. Saunders; 1995. p. 449-58.  2 Hughes J. Melanoma of the umbilicus: a case report. J Ir Med Assoc. 1963;53:94-8. PMID: 14050217.187Umbilical melanoma histopathology  3 Steck WD, Helwig EB. Tumors of the umbilicus. Cancer. 1965;18:907-15. PMID: 14308240.  4 Colonna MR, Giovannini UM, Sturniolo G, Colonna U. The umbilicus: a rare site for melanoma. Clinical considerations in two cases. Scand J Plast Reconstr Hand Surg. 1999;33:449-52. PMID: 10614756.  5 Meine JG, Bailin PL. Primary melanoma of the umbilicus: report of a case and review of the relevant anatomy. Dermatol Surg. 2003;29:404-7. PMID: 12656822.  6 Campos-Munoz L, Quesada-Cortes A, Ruiz E, Casado M, Pizarro A. Primary melanoma of the umbilicus appearing as omphalitis. Clin Exp Dermatol. 2007;32:322-4. PMID: 17335551.  7 Mangas C, Romani J, Munoz C, Luelmo J. Navel melanoma: not always easy to detect, not always difficult to remove. Der-matol Online J. 2008;14:20. PMID: 19094858.  8 Cecchi R, Pavesi M, Buralli L, Rapicano V, De Gaudio C. Primary umbilical melanoma. Australas J Dermatol. 2009;50:220-2. PMID: 19659989.  9 Zaccagna A, Siatis D, Pisacane A, Giacone E, Picciotto F. Surgical treatment of primary melanoma of the umbilicus with sentinel lymph node biopsy and plastic reconstruction: Case report and review of the literature. Eur J Surg Oncol. 2011;37:233-6. PMID: 20961729.10 Papalas JA, Selim MA. Metastatic vs malignant neoplasms affecting the umbilicus: clinicopathologic features of 77 tu-mors. Ann Diagn Pathol. 2011;15:237-42. PMID: 21419680.11 Navysany S, Daigeler A, Dippel E, Loser C. Reconstruction of the umbilicus after malignant melanoma. J Dtsch Dermatol Ges. 2013;11:462-4. PMID: 23464794.12 Dessy LA, Maruccia M, Romanzi A, Onesti MG. Melanoma of the umbilicus: an incidental diagnosis during fat-harvesting donor-site selection. Aesth Plast Surg. 2013;37:489-90. PMID: 23435508.13 Song Y, Xu D, Sun L, Ding K, Hu Y, Yuan Y. Diagnosis and management of primary umbilical melanoma with omphalitis features. Case Rep Oncol. 2013;6:154-7. PMID: 23626553.14 Clark WH, From L, Bernardino EA, Mihm MC. The histo-genesis and biologic behavior of primary human malignant melanomas of the skin. Cancer Research. 1969;29:705-26. PMID: 5773814.15 Ross MI, Balch CM. Surgical treatment of primary melano-ma. In: Balch CM, Houghton AN, Sober AJ. Soong S, eds. Cutaneous Melanoma, 3rd ed. St Louis: Quality Medical Pub-lishing Inc; 1998. p. 142-52.

Melanoma of the Umbilicus: A Patient Report, Precaution in Operative Strategy, and the First Histopathological Review of Published Cases

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Melanoma of the Umbilicus: A Patient Report, Precaution in Operative Strategy, and the First Histopathological Review of Published Cases

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