Correspondence Clinical Letter
Clinical Letter An ulcerated giant pilomatricoma mimicking malignancy
DOI: 10.1111/ddg.12538
Dear Editors, Pilomatricoma is a benign cutaneous tumor which typically presents as a subcutaneous nodule sized 0.5 to 3 cm on the head, neck or upper extremities [1]. We present a patient with a giant ulcerated pilomatricoma on the right arm, which clinically appeared as a malignant tumor but showed a completely benign histopathology. A 75-year-old woman presented with a 4-year history of enlarging painless mass on her right forearm. The lesion had started growing in size several months ago and eventually attained the current size. Examination revealed a firm, solitary, ulcerated and indurated 3.2 × 5.5 cm tumor on the right forearm (Figure 1). Systemic examination and routine blood tests were normal. Histopathological examination of the incisional biopsy showed a well-demarcated lobular epithelial tumor, consisting of basaloid cells and eosinophilic shadow cells with focal areas of calcium deposits in the dermis (Figure 2). There were no pronounced cytological and nuclear atypia; the tumor showed a low mitotic activity (Figure 3). These histopathological findings were compatible with pilomatricoma. Pilomatricoma was first defined as a benign tumor originating from the sebaceous glands in 1880 by Malherbe and Chenantais who interpreted the tumor as a calcifying
Figure 1 Ulcerated, indurated, non-tender, 3.2cm × 5.5-cm tumor on the forearm.
e pithelioma. Forbis and Helwig in 1961 demonstrated that the tumor originated from the hair matrix and introduced the term pilomatricoma [1]. The lesions are most commonly seen on the head and neck region followed by the upper extremities. Classically, the tumor presents as a solitary, slow-growing, firm, small dermal nodule with normal or blue-black overlying skin. Multiple and familial lesions have been reported in association with conditions such as myotonic dystrophy, Gardner syndrome, Rubinstein-Taybi syndrome and Turner syndrome [2, 3]. The tumor is common in children, and more than 60 % of cases develop in the first two decades of life [1, 2]. Uncommon clinical-pathological variants of pilomatricoma have been reported, such as bullous, anetodermic, exophytic,
Figure 2 Skin biopsy showing islands of eosinophilic shadow cells and basaloid cells with areas of calcification (H&Ex100).
Figure 3 Close-up view of basaloid cells showing scant mitoses without atypia (H&Ex400).
© 2015 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2015/1304
329
Correspondence Clinical Letter
erforating/ulcerated, giant and lymphangiectatic [4]. The p size of the tumor usually ranges from 0.5 to 6 cm. Lesions measuring greater than 5 cm are defined as giant pilomatricomas [5]. In 1974, Krausen reported the first giant pilomatricoma with a 7 × 6 × 5 cm of size located in the parotid region. Giant pilomatricomas are generally located on the head, neck and upper extremities [1]. Because pilomatricomas may resemble other skin tumors such as epidermoid cysts, dermatofibrosarcoma protuberans, calcinosis cutis, cutaneous metastases, calcified hemangioma, giant dermatofibroma or nodular basal cell carcinoma, histopathological examination is crucial for definitive diagnosis [6]. The histopathology of pilomatricoma shows a well- circumscribed, deep dermal or dermal-subcutaneous tumor formed by basaloid cells that gradually lose their nuclei and mingle with the eosinophilic shadow cells that show ghosts of epithelial cells. Calcification may be seen in shadow cell regions along with foreign body giant cell reaction to keratin [7]. Malignant transformation with cell necrosis and mitotic figures have been documented in pilomatricoma [6]. In this case pilomatrical carcinoma was excluded because of absence of high mitotic activity and nuclear atypia. Total surgical excision is the treatment of choice for pilomatricomas; recurrences are rare [4]. In conclusion, although pilomatricoma is a commonly seen tumor, there are no clinical criteria for diagnosis. Since it may be confused with other tumors of the skin such as sarcomas and cutaneous metastases, histopathological examination is critical.
Ilteris Oguz Topal1, Ralfi Singer 1, Ozgur Emek Kocaturk Goncu1, Yunus Topal2, Enver Yarıkkaya3, Ilknur Mansuroglu Sahin3
Conflict of interest None.
5
(1) Department of Dermatology, Okmeydani Training and Research Hospital, Istanbul, Turkey (2) Department of General Surgery, Gaziosmanpa¸sa Taksim Training and Research Hospital, Istanbul, Turkey (3) Department of Pathology, Okmeydani Training and Research Hospital, Istanbul, Turkey
Correspondence to Ilteris Oguz Topal Department of Dermatology, Okmeydani Training and R esearch Hospital, Istanbul, Turkey E-mail: [email protected]
References 1
2
3
4
6
7
330
Goktay F, Mansur AT, Aydingoz IE et al. A rare presentation of giant pilomatricoma located on the back. Dermatol Surg 2007; 33: 596–600. Nakai K, Yoneda K, Maeda R et al. Giant pilomatricoma and psoriasis vulgaris with myotonic dystrophy. Eur J Dermatol 2009; 19: 507–8. Wolff CR, Brehmer F, Lockmann A et al. Rapidly growing bluered nodule on the cheek of a 4-year-old boy. Pilomatricoma. J Dtsch Dermatol Ges 2014; 12: 432–4. Ciralik H, Coban YK, Arican O. A case of perforating pilomatricoma. J Dermatol 2006; 33: 394–8. Stefano P, Apa S, Balancini B. Ulcerated giant pilomatrixoma in a child. Actas Dermosifiliogr 2013; 104: 924–5. Turhan-Haktanir N, Demir Y, Sahin O et al. Pilomatricoma: a review of six pediatric cases with nine lesions. Turk J Pediatr 2009; 51: 44–8. Nadershah M, Alshadwi A, Salama A. Recurrent giant pilomatrixoma of the face: a case report and review of the literature. Case Rep Dent 2012; 2012: 197–273.
© 2015 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2015/1304
Clinical Letter An ulcerated giant pilomatricoma mimicking malignancy
DOI: 10.1111/ddg.12538
Dear Editors, Pilomatricoma is a benign cutaneous tumor which typically presents as a subcutaneous nodule sized 0.5 to 3 cm on the head, neck or upper extremities [1]. We present a patient with a giant ulcerated pilomatricoma on the right arm, which clinically appeared as a malignant tumor but showed a completely benign histopathology. A 75-year-old woman presented with a 4-year history of enlarging painless mass on her right forearm. The lesion had started growing in size several months ago and eventually attained the current size. Examination revealed a firm, solitary, ulcerated and indurated 3.2 × 5.5 cm tumor on the right forearm (Figure 1). Systemic examination and routine blood tests were normal. Histopathological examination of the incisional biopsy showed a well-demarcated lobular epithelial tumor, consisting of basaloid cells and eosinophilic shadow cells with focal areas of calcium deposits in the dermis (Figure 2). There were no pronounced cytological and nuclear atypia; the tumor showed a low mitotic activity (Figure 3). These histopathological findings were compatible with pilomatricoma. Pilomatricoma was first defined as a benign tumor originating from the sebaceous glands in 1880 by Malherbe and Chenantais who interpreted the tumor as a calcifying
Figure 1 Ulcerated, indurated, non-tender, 3.2cm × 5.5-cm tumor on the forearm.
e pithelioma. Forbis and Helwig in 1961 demonstrated that the tumor originated from the hair matrix and introduced the term pilomatricoma [1]. The lesions are most commonly seen on the head and neck region followed by the upper extremities. Classically, the tumor presents as a solitary, slow-growing, firm, small dermal nodule with normal or blue-black overlying skin. Multiple and familial lesions have been reported in association with conditions such as myotonic dystrophy, Gardner syndrome, Rubinstein-Taybi syndrome and Turner syndrome [2, 3]. The tumor is common in children, and more than 60 % of cases develop in the first two decades of life [1, 2]. Uncommon clinical-pathological variants of pilomatricoma have been reported, such as bullous, anetodermic, exophytic,
Figure 2 Skin biopsy showing islands of eosinophilic shadow cells and basaloid cells with areas of calcification (H&Ex100).
Figure 3 Close-up view of basaloid cells showing scant mitoses without atypia (H&Ex400).
© 2015 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2015/1304
329
Correspondence Clinical Letter
erforating/ulcerated, giant and lymphangiectatic [4]. The p size of the tumor usually ranges from 0.5 to 6 cm. Lesions measuring greater than 5 cm are defined as giant pilomatricomas [5]. In 1974, Krausen reported the first giant pilomatricoma with a 7 × 6 × 5 cm of size located in the parotid region. Giant pilomatricomas are generally located on the head, neck and upper extremities [1]. Because pilomatricomas may resemble other skin tumors such as epidermoid cysts, dermatofibrosarcoma protuberans, calcinosis cutis, cutaneous metastases, calcified hemangioma, giant dermatofibroma or nodular basal cell carcinoma, histopathological examination is crucial for definitive diagnosis [6]. The histopathology of pilomatricoma shows a well- circumscribed, deep dermal or dermal-subcutaneous tumor formed by basaloid cells that gradually lose their nuclei and mingle with the eosinophilic shadow cells that show ghosts of epithelial cells. Calcification may be seen in shadow cell regions along with foreign body giant cell reaction to keratin [7]. Malignant transformation with cell necrosis and mitotic figures have been documented in pilomatricoma [6]. In this case pilomatrical carcinoma was excluded because of absence of high mitotic activity and nuclear atypia. Total surgical excision is the treatment of choice for pilomatricomas; recurrences are rare [4]. In conclusion, although pilomatricoma is a commonly seen tumor, there are no clinical criteria for diagnosis. Since it may be confused with other tumors of the skin such as sarcomas and cutaneous metastases, histopathological examination is critical.
Ilteris Oguz Topal1, Ralfi Singer 1, Ozgur Emek Kocaturk Goncu1, Yunus Topal2, Enver Yarıkkaya3, Ilknur Mansuroglu Sahin3
Conflict of interest None.
5
(1) Department of Dermatology, Okmeydani Training and Research Hospital, Istanbul, Turkey (2) Department of General Surgery, Gaziosmanpa¸sa Taksim Training and Research Hospital, Istanbul, Turkey (3) Department of Pathology, Okmeydani Training and Research Hospital, Istanbul, Turkey
Correspondence to Ilteris Oguz Topal Department of Dermatology, Okmeydani Training and R esearch Hospital, Istanbul, Turkey E-mail: [email protected]
References 1
2
3
4
6
7
330
Goktay F, Mansur AT, Aydingoz IE et al. A rare presentation of giant pilomatricoma located on the back. Dermatol Surg 2007; 33: 596–600. Nakai K, Yoneda K, Maeda R et al. Giant pilomatricoma and psoriasis vulgaris with myotonic dystrophy. Eur J Dermatol 2009; 19: 507–8. Wolff CR, Brehmer F, Lockmann A et al. Rapidly growing bluered nodule on the cheek of a 4-year-old boy. Pilomatricoma. J Dtsch Dermatol Ges 2014; 12: 432–4. Ciralik H, Coban YK, Arican O. A case of perforating pilomatricoma. J Dermatol 2006; 33: 394–8. Stefano P, Apa S, Balancini B. Ulcerated giant pilomatrixoma in a child. Actas Dermosifiliogr 2013; 104: 924–5. Turhan-Haktanir N, Demir Y, Sahin O et al. Pilomatricoma: a review of six pediatric cases with nine lesions. Turk J Pediatr 2009; 51: 44–8. Nadershah M, Alshadwi A, Salama A. Recurrent giant pilomatrixoma of the face: a case report and review of the literature. Case Rep Dent 2012; 2012: 197–273.
© 2015 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2015/1304
