Letters to the Editor
Case of pigmented epithelioid melanocytoma with lymph node metastases Dear Editor, Pigmented epithelioid melanocytoma (PEM) is a relatively new and rare type of melanocytic tumor that is composed of epithelioid, pleomorphic, atypical cells with heavy pigmentation.1 Despite a high rate of lymphatic involvement, PEM is believed to have a favorable prognosis compared with melanoma.2 Herein, we report a case of PEM that was shown to have multiple lymph node (LN) metastases. A 4-year-old Japanese boy presented with a darkly pigmented tumor on his right buttock. He had been born with a blue macule on the right buttock that had rapidly become enlarged and elevated in the past 6 months. The physical examination revealed a 3-cm dome-shaped, blue-black tumor with ulceration (Fig. 1a). Several pigmented papules surrounded the tumor, indicating satellitosis. Because of its relatively rapid growth, large size and satellitosis, we suspected the tumor to be a malignant blue nevus. Therefore, we excised it with wide margins and performed a sentinel LN biopsy. Histological examination revealed a darkly pigmented tumor partly infiltrated into the subcutis (Fig. 1b). The tumor cells were composed of epithelioid, pleomorphic, atypical cells with heavy pigmentation (Fig. 1c). No mitoses were found. Immunohistochemically, the tumor cells were stained positive for HMB-45 and S-100. According to these results, we diagnosed this tumor as a PEM. The patient underwent LN dissection of his right groin because two of the three sentinel LN carried metastases (Fig. 1d,e). The patient is still alive without relapse 42 months after the treatment. Several cases of PEM have been reported2,3 since Zembowicz et al.1 proposed the concept of a low-grade melanocytic tumor with metastatic potential indistinguishable from animal-type melanoma and epithelioid blue nevus. PEM can occur sporadically or in patients with Carney complex.1 Carney complex is a familial multiple neoplasia and lentiginous syndrome in which multiple PEM develop as a result of a mutation of the protein kinase A regulatory subunit 1a (PRKAR1a) gene. However, although loss of PRKAR1a expression in PEM may be useful diagnostic information, it is not essential because 18% of sporadic PEM express PRKAR1a4 as our case (Fig. 1f). In our case, we diagnosed PEM rather than malignant blue nevus because the pathological features of the lesion such as low mitotic activity and epithelioid tumor cells with massive pigmentation were consistent with the proposed criteria for PEM.3 However, some investigators consider PEM a flawed concept.5 The biggest issue to be addressed is whether PEM is a malignant or a benign tumor. Mandal et al.2 reported that eight
(a)
(b)
(c)
(d)
(e)
(f)
Figure 1. (a) Blue-black tumor with ulceration located on the right buttock. Note the presence of several pigmented papules around the tumor. (b) Hematoxylin–eosin (HE) stain, scanning magnification. Darkly pigmented tumor located in the dermis with partly infiltrative growth into the subcutis. (c) HE stain, original magnification 9400. The tumor cells were heavily pigmented. (d) Melanin-bleached specimen with HE staining, original magnification 9400. The tumor cells were composed of epithelioid cells. Mitosis was rarely found. (e) Staining for protein kinase A regulatory subunit 1a (PRKAR1a), original magnification 9200. Most of the tumor cells stained moderately positive for PKAR1a. (f) Staining for HMB-45 in a sentinel lymph node (LN), original magnification 9400. HMB-45-positive tumor cells were aggregated in the cortex of the LN (arrowheads). Green-stained cells represent melanin deposition due to Giemsa staining.
Correspondence: Yasuhiro Fujisawa, M.D., Ph.D., Department of Dermatology, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki 305-8575, Japan. Email: [email protected]
452
© 2014 Japanese Dermatological Association
Letters to the Editor
of 26 PEM patients (31%) had LN metastasis; however, none of the patients died of the disease during a median follow-up period of 67 months. Thus, the current understanding of PEM is that it may have the potential to metastasize via the lymphatic channels, but it is not fatal. However, we recommend sentinel LN biopsy because the concept of PEM has yet to be well established.
ACKNOWLEDGMENTS: We are grateful to Ms Flaminia Miyamasu for her excellent proofreading. This work was partly supported by the National Cancer Center Research and Development Fund (23-A-22). There was no other source of funding. CONFLICT OF INTEREST:
None.
Yasuhiro FUJISAWA, Hiroshi MARUYAMA, Jun-ichi FURUTA, Yoshiyuki ISHII, Yasuhiro KAWACHI, Manabu FUJIMOTO
doi: 10.1111/1346-8138.12482
REFERENCES 1 Zembowicz A, Carney JA, Mihm MC. Pigmented epithelioid melanocytoma: a low-grade melanocytic tumor with metastatic potential indistinguishable from animal-type melanoma and epithelioid blue nevus. Am J Surg Pathol 2004; 28: 31–40. 2 Mandal RV, Murali R, Lundquist KF et al. Pigmented epithelioid melanocytoma: favorable outcome after 5-year follow-up. Am J Surg Pathol 2009; 33: 1778–1782. 3 Ito K, Mihm MC. Pigmented epithelioid melanocytoma: report of first Japanese cases previously diagnosed as cellular blue nevus. J Cutan Pathol 2009; 36: 439–443. 4 Zembowicz A, Knoepp SM, Bei T et al. Loss of expression of protein kinase a regulatory subunit 1alpha in pigmented epithelioid melanocytoma but not in melanoma or other melanocytic lesions. Am J Surg Pathol 2007; 31: 1764–1775. 5 Milette F. Pigmented epithelioid melanocytoma: report of first Japanese cases previously diagnosed as cellular blue nevus. J Cutan Pathol 2009; 36: 1027–1028.
Department of Dermatology, University of Tsukuba, Tsukuba, Japan
Involuted facial infantile hemangioma with fatty replacement successfully treated with surgery Dear Editor, Infantile hemangioma (IH) is a vascular tumor, and its hallmark is rapid growth during the first year of life, followed by slow regression during early childhood.1,2 Therefore, there have been few reports describing a long-term follow up over 10 years and histological examinations of involuted IH. A 3-month-old female infant presented to us with rapidly growing reddish tumors on the right cheek. Physical examination revealed multiple, reddish tumors with a subcutaneous mass covering almost the entire right cheek (Fig. 1a). Based on clinical findings, the lesion was diagnosed as an IH consisting of red superficial and deep subcutaneous components, namely the mixed type of IH. Despite the repeated pulsed dye laser treatments, the lesion had gradually increased in size and bluish discoloration of the skin appeared (Fig. 1b). At 8 and 9 months of age, intralesional injection of corticosteroid (triamcinolone acetonide; Kenacort-A [Bristol-Myers Squibb, Tokyo, Japan]: first, 30 mg; second, 50 mg) was carried out, although the immediate effect was minimal. Pulsed dye laser irradiation was performed at an interval of 3 months until 3 years of age. The lesion diminished very slowly over 10 years. A soft, subcutaneous mass covered partly with telangiectatic, atrophic skin remained on her right cheek at 12 years of age (Fig. 1c). Magnetic resonance imaging (MRI) showed
thickened subcutaneous fat tissue in the right cheek (Fig. 1d). To improve her facial contour and aesthetics, we resected excessive fat tissue and atrophic skin of her right cheek using an incision along the nasolabial fold under general anesthesia. Histologically, the resected tissue was composed of mature adipose tissue including a few mature blood vessels and fibrous tissue (Fig. 1e). A good aesthetic result was achieved (Fig. 1f). Fifty percent of IH completely resolve by the age of 5 years and 90% by the age of 9 years.2 Some patients suffer from cosmetic sequelae caused by residual angioma, telangiectasia and scarring. There have been few reports describing involuted IH with replacement by excess adipose tissue. Chan et al. reported three cases of periorbital IH that showed a baggy appearance of the eyelids because of excess fat tissue, treated by resection of the excess fat tissue.3 Large or deep IH which may cause serious complications or permanent disfigurement need to be treated. Various treatments exist, including surgical excision, pulsed dye laser, corticosteroid therapy (topical application, intralesional injection, systemic medication) and the b-adrenergic receptor antagonist propranolol.1,2 Nowadays, propranolol is considered the first choice of the treatment of IH. Unfortunately, we could not use of propranolol, because the use of propranolol is not approved in Japan. Pulsed dye laser is effective mainly for superficial
Correspondence: Akihiko Uchiyama, M.D., Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma 371-8511, Japan. Email: [email protected]
© 2014 Japanese Dermatological Association
453
Case of pigmented epithelioid melanocytoma with lymph node metastases Dear Editor, Pigmented epithelioid melanocytoma (PEM) is a relatively new and rare type of melanocytic tumor that is composed of epithelioid, pleomorphic, atypical cells with heavy pigmentation.1 Despite a high rate of lymphatic involvement, PEM is believed to have a favorable prognosis compared with melanoma.2 Herein, we report a case of PEM that was shown to have multiple lymph node (LN) metastases. A 4-year-old Japanese boy presented with a darkly pigmented tumor on his right buttock. He had been born with a blue macule on the right buttock that had rapidly become enlarged and elevated in the past 6 months. The physical examination revealed a 3-cm dome-shaped, blue-black tumor with ulceration (Fig. 1a). Several pigmented papules surrounded the tumor, indicating satellitosis. Because of its relatively rapid growth, large size and satellitosis, we suspected the tumor to be a malignant blue nevus. Therefore, we excised it with wide margins and performed a sentinel LN biopsy. Histological examination revealed a darkly pigmented tumor partly infiltrated into the subcutis (Fig. 1b). The tumor cells were composed of epithelioid, pleomorphic, atypical cells with heavy pigmentation (Fig. 1c). No mitoses were found. Immunohistochemically, the tumor cells were stained positive for HMB-45 and S-100. According to these results, we diagnosed this tumor as a PEM. The patient underwent LN dissection of his right groin because two of the three sentinel LN carried metastases (Fig. 1d,e). The patient is still alive without relapse 42 months after the treatment. Several cases of PEM have been reported2,3 since Zembowicz et al.1 proposed the concept of a low-grade melanocytic tumor with metastatic potential indistinguishable from animal-type melanoma and epithelioid blue nevus. PEM can occur sporadically or in patients with Carney complex.1 Carney complex is a familial multiple neoplasia and lentiginous syndrome in which multiple PEM develop as a result of a mutation of the protein kinase A regulatory subunit 1a (PRKAR1a) gene. However, although loss of PRKAR1a expression in PEM may be useful diagnostic information, it is not essential because 18% of sporadic PEM express PRKAR1a4 as our case (Fig. 1f). In our case, we diagnosed PEM rather than malignant blue nevus because the pathological features of the lesion such as low mitotic activity and epithelioid tumor cells with massive pigmentation were consistent with the proposed criteria for PEM.3 However, some investigators consider PEM a flawed concept.5 The biggest issue to be addressed is whether PEM is a malignant or a benign tumor. Mandal et al.2 reported that eight
(a)
(b)
(c)
(d)
(e)
(f)
Figure 1. (a) Blue-black tumor with ulceration located on the right buttock. Note the presence of several pigmented papules around the tumor. (b) Hematoxylin–eosin (HE) stain, scanning magnification. Darkly pigmented tumor located in the dermis with partly infiltrative growth into the subcutis. (c) HE stain, original magnification 9400. The tumor cells were heavily pigmented. (d) Melanin-bleached specimen with HE staining, original magnification 9400. The tumor cells were composed of epithelioid cells. Mitosis was rarely found. (e) Staining for protein kinase A regulatory subunit 1a (PRKAR1a), original magnification 9200. Most of the tumor cells stained moderately positive for PKAR1a. (f) Staining for HMB-45 in a sentinel lymph node (LN), original magnification 9400. HMB-45-positive tumor cells were aggregated in the cortex of the LN (arrowheads). Green-stained cells represent melanin deposition due to Giemsa staining.
Correspondence: Yasuhiro Fujisawa, M.D., Ph.D., Department of Dermatology, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki 305-8575, Japan. Email: [email protected]
452
© 2014 Japanese Dermatological Association
Letters to the Editor
of 26 PEM patients (31%) had LN metastasis; however, none of the patients died of the disease during a median follow-up period of 67 months. Thus, the current understanding of PEM is that it may have the potential to metastasize via the lymphatic channels, but it is not fatal. However, we recommend sentinel LN biopsy because the concept of PEM has yet to be well established.
ACKNOWLEDGMENTS: We are grateful to Ms Flaminia Miyamasu for her excellent proofreading. This work was partly supported by the National Cancer Center Research and Development Fund (23-A-22). There was no other source of funding. CONFLICT OF INTEREST:
None.
Yasuhiro FUJISAWA, Hiroshi MARUYAMA, Jun-ichi FURUTA, Yoshiyuki ISHII, Yasuhiro KAWACHI, Manabu FUJIMOTO
doi: 10.1111/1346-8138.12482
REFERENCES 1 Zembowicz A, Carney JA, Mihm MC. Pigmented epithelioid melanocytoma: a low-grade melanocytic tumor with metastatic potential indistinguishable from animal-type melanoma and epithelioid blue nevus. Am J Surg Pathol 2004; 28: 31–40. 2 Mandal RV, Murali R, Lundquist KF et al. Pigmented epithelioid melanocytoma: favorable outcome after 5-year follow-up. Am J Surg Pathol 2009; 33: 1778–1782. 3 Ito K, Mihm MC. Pigmented epithelioid melanocytoma: report of first Japanese cases previously diagnosed as cellular blue nevus. J Cutan Pathol 2009; 36: 439–443. 4 Zembowicz A, Knoepp SM, Bei T et al. Loss of expression of protein kinase a regulatory subunit 1alpha in pigmented epithelioid melanocytoma but not in melanoma or other melanocytic lesions. Am J Surg Pathol 2007; 31: 1764–1775. 5 Milette F. Pigmented epithelioid melanocytoma: report of first Japanese cases previously diagnosed as cellular blue nevus. J Cutan Pathol 2009; 36: 1027–1028.
Department of Dermatology, University of Tsukuba, Tsukuba, Japan
Involuted facial infantile hemangioma with fatty replacement successfully treated with surgery Dear Editor, Infantile hemangioma (IH) is a vascular tumor, and its hallmark is rapid growth during the first year of life, followed by slow regression during early childhood.1,2 Therefore, there have been few reports describing a long-term follow up over 10 years and histological examinations of involuted IH. A 3-month-old female infant presented to us with rapidly growing reddish tumors on the right cheek. Physical examination revealed multiple, reddish tumors with a subcutaneous mass covering almost the entire right cheek (Fig. 1a). Based on clinical findings, the lesion was diagnosed as an IH consisting of red superficial and deep subcutaneous components, namely the mixed type of IH. Despite the repeated pulsed dye laser treatments, the lesion had gradually increased in size and bluish discoloration of the skin appeared (Fig. 1b). At 8 and 9 months of age, intralesional injection of corticosteroid (triamcinolone acetonide; Kenacort-A [Bristol-Myers Squibb, Tokyo, Japan]: first, 30 mg; second, 50 mg) was carried out, although the immediate effect was minimal. Pulsed dye laser irradiation was performed at an interval of 3 months until 3 years of age. The lesion diminished very slowly over 10 years. A soft, subcutaneous mass covered partly with telangiectatic, atrophic skin remained on her right cheek at 12 years of age (Fig. 1c). Magnetic resonance imaging (MRI) showed
thickened subcutaneous fat tissue in the right cheek (Fig. 1d). To improve her facial contour and aesthetics, we resected excessive fat tissue and atrophic skin of her right cheek using an incision along the nasolabial fold under general anesthesia. Histologically, the resected tissue was composed of mature adipose tissue including a few mature blood vessels and fibrous tissue (Fig. 1e). A good aesthetic result was achieved (Fig. 1f). Fifty percent of IH completely resolve by the age of 5 years and 90% by the age of 9 years.2 Some patients suffer from cosmetic sequelae caused by residual angioma, telangiectasia and scarring. There have been few reports describing involuted IH with replacement by excess adipose tissue. Chan et al. reported three cases of periorbital IH that showed a baggy appearance of the eyelids because of excess fat tissue, treated by resection of the excess fat tissue.3 Large or deep IH which may cause serious complications or permanent disfigurement need to be treated. Various treatments exist, including surgical excision, pulsed dye laser, corticosteroid therapy (topical application, intralesional injection, systemic medication) and the b-adrenergic receptor antagonist propranolol.1,2 Nowadays, propranolol is considered the first choice of the treatment of IH. Unfortunately, we could not use of propranolol, because the use of propranolol is not approved in Japan. Pulsed dye laser is effective mainly for superficial
Correspondence: Akihiko Uchiyama, M.D., Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma 371-8511, Japan. Email: [email protected]
© 2014 Japanese Dermatological Association
453
