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Metastasizing dermatofibroma in lung
Annals of Diagnostic Pathology 11 (2007) 64 – 67
Metastasizing dermatofibroma in lung MiJin Gu, MD, MSa,4, KyungRak Sohn, MD, PhDa, DongJa Kim, MD, PhDa, ByungHo Kim, MD, PhDb b
a Department of Pathology, Daegu Fatima Hospital, Daegu 701-600, Korea Department of Thoracic Surgery, Daegu Fatima Hospital, Daegu 701-600, Korea
Abstract
Benign dermatofibroma is very common skin tumor and can very rarely metastasize. We report a case of metastasizing dermatofibroma on a 36-year-old woman who presented multiple bilateral lung nodules. She underwent incisional biopsy for cellular dermatofibroma of the right shoulder 7 years ago. Chest computed tomographic scanning shows multiple nodules in both lung fields. Segmental and wedge resections were done. Grossly, the masses were hemorrhagic cysts. Microscopically, there were dilated cystic airspaces. The airspaces were lined by respiratory and metaplastic squamous epithelium with underlying layers of fibrohistiocytic spindle cells with storiform and fascicular pattern. The tumor cells stained for CD68 and CD10. The lung mass shows same histologic features with skin lesion. D 2007 Elsevier Inc. All rights reserved.
Keywords:
Dermatofibroma; Lung; Cyst; Metastasis
1. Introduction Benign dermatofibroma is a very common skin tumor and may recur. However, metastasis of dermatofibroma is a very uncommon event [1- 3]. To the best of our knowledge, 12 cases were reported in the English literature since the first description by Joseph et al [2] in 1990. On the observations of previous published cases, the tumors occur in young male adults and present as multiple cavitary or cystic lesions (Table 1). We report a rare case of metastasizing dermatofibroma in a 36-year-old woman without respiratory symptom and review of literatures. 2. Case report A 36-year-old woman visited our hospital for mental change and deviation of tongue. She had a history of diabetes mellitus, hypertension, and hyperthyroidism. At the time of initial examination, she had no respiratory symptom. She had excised cutaneous shoulder nodule 7 years ago. The mass was a cellular dermatofibroma. We could not review the previous skin slide because the slides and blocks were 4 Corresponding author. Tel.: +82 53 940 7272; fax: +82 53 940 7273. E-mail address: [email protected] (M.J. Gu). 1092-9134/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.anndiagpath.2005.12.001
scrapped. Chest computed tomographic (CT) scanning shows multiple discrete, margined round nodules in both lung fields (Fig. 1). Wedge and segmental resections were done. Grossly, hemorrhagic cystic masses were noted: 3 in the right upper lobe, 1 in the right middle lobe, 2 in the right lower lobe, 5 in the left upper lobe, and 4 in the left lower lobe of lung. The size of cysts was 1.2 to 3.9 cm. They were multilocular cysts containing chocolate-colored hemorrhagic material, similar to endometriosis of ovary (Fig. 2). Microscopically, there were dilated cystic airspaces. The airspaces were lined by respiratory epithelium with underlying layers of tumor cells. The tumor cells were composed of plump to spindled fibrohistiocytic cells arranged in storiform growth pattern (Fig. 3). There were no cellular pleomorphism, atypical mitosis, and necrosis. Foamy macrophages, hemosiderin depositions, and cholesterol clefts were observed (Fig. 4). On immunohistochemistry, the tumor cells were stained for CD68 (514H12, Predilution; Ventana, Tucson, AZ) and CD10 (56C6, Predilution; Ventana), but negative on CD34 (QBEnd/10, Predilution; Ventana), CD117 (Polyclonal, 1:50; Dako, Carpinteria, CA) and estrogen receptor (6F11, Predilution; Ventana) was noted (Fig. 5). After the operation of lung, we examined the previous skin lesion and found small remaining cellular dermatofibroma due to previous incomplete excision. The
M.J. Gu et al. / Annals of Diagnostic Pathology 11 (2007) 64 – 67
65
Table 1 Clinical findings of metastatic dermatofibroma in lung Case
Sex
Age
Respiratory symptom
Radiologic or pathological finding
Previous cutaneous lesion
Follow-up (y)
1 2 3 4
M M M M
38 54 35 29
Bilateral Bilateral Bilateral Multiple
Not identified CHF (excluded 23 y ago) Not identified CHF (10 y ago on back)
Alive with disease at 2 y Alive with disease at 2 y Alive with disease at 2 y Not known
5 6
M M
65 30
Bilateral multiple nodular opacities Bilateral nodular opacities
Not identified Recurrent DH (5 y ago on back)
Alive with disease at 2 y Alive with disease at 2 y
7 8 9 10 11 12 13
M F M M M M F
25 30 19 40 33 33 36
Hemoptysis Hemoptysis Not known Pneumonia, pneumothorax Asymptomatic Dyspnea, pneumothorax Hemoptysis Pneumothorax Not known Not known Fatigue No No
Bilateral diffuse cavities Bilateral cystic lesions Cystic lesion on gross finding Cystic lesion on gross finding Multiple masses, not cystic Bilateral multiple cystic lesions Bilateral multiple cystic lesions
Not identified DFP (17 y ago) CHF (1.5 y ago) CHF (7 y ago) CFH (2 y ago) CFH (9 y ago) CFH (7 y ago)
Alive with disease Not known Alive with disease Alive with disease Alive with disease Alive with disease Alive with disease
many nodular and cavitating opacity with many nodules cystic lung nodules bilateral cavitary lesions
at 2 y at 2 y at 2 y at 6 y at 1 y
M, male; F, female; CFH, Cellular fibrous histiocytoma; DF, Dermatofibroma; DFP, Dermatofibrosarcoma protuberans.
mass was 0.4 0.3 cm in size and showed the same histologic features with lung lesion (Fig. 6).
sarcoma [1,6]. We had difficulty of diagnosis on initial small wedge resection because the tumor cells showed positive for CD10 and negative for CD68. And small uterine mass was
3. Discussion Metastasis of dermatofibroma is a very uncommon event [1- 3]. Most previously reported cases were metastasis of cellular dermatofibroma [4,5]. The tumors often occur in young male adults, and they had excision cellular dermatofibroma of skin 1.5 to 23 years ago before lung disease [4]. Gillou et al [6] described that relatively large size, high cellularity, aneurysmal changes, marked cellular pleomorphism, high mitotic figures, tumor necrosis, and local recurrence are risk factors for metastasis. Differential diagnosis includes lymphangioleiomyomatosis, dermatofibroma, angiomatoid fibrous histiocytoma, dermatofibrosarcoma protuberans, solitary fibrous tumor in lung, endometriosis, and metastatic endometrial stromal
Fig. 1. Well-margined multiple masses present in right lung on chest CT scanning.
Fig. 2. Grossly, the lung masses are hemorrhagic cysts (from right: upper, middle, and lower lobe mass).
Fig. 3. Sections of the lung show the wall of cyst is lined by respiratory epithelium with underlying layers of spindle cells.
66
M.J. Gu et al. / Annals of Diagnostic Pathology 11 (2007) 64 – 67
Fig. 4. The tumor cells composed of fibrohistiocytic spindle cells with storiform and fascicular pattern. Foam cells, inflammatory cells, and hemosiderin depositions are mixed.
detected in abdominal sonogram. However, on whole masses excision, the tumor cells were positive on CD68 and CD10, and the uterine mass was a leiomyoma. Lymphangioleiomyomatosis should be excluded because it
Fig. 6. Sections of skin show remaining dermatofibroma in reticular dermis.
occurs women of childbearing age. However, the spindle tumor cells of lymphangioleiomyomatosis were positive on smooth muscle actin and HMB-45 [6]. Angiomatoid fibrous histiocytoma can be excluded in our case because the tumors had storiform pattern and lacked peripheral cuffing of lymphocytes and plasma cells (a fibrous pseudocapsule)
Fig. 5. On immunohistochemical staining, the tumor cells stain for CD68 and CD10. The lining epithelial cells stain for thyroid transcription factor (TTF)-1.
M.J. Gu et al. / Annals of Diagnostic Pathology 11 (2007) 64 – 67
[1,6]. Dermatofibrosarcoma was much less dermarcated and showed more uniform spindle tumor cells, and the tumor cells were positive for CD34 [6]. Osborn et al [4] reported a review of 10 published cases of metastasizing dermatofibroma. The masses were presented as cystic fibrohistiocytic tumor in the lung. Our case also showed multiple hemorrhagic cysts as endometriosis of ovary. The masses were presented with multiple bilateral cystic lung lesions or nodular cavitary lesions on imaging study (Table 1). It is uncertain, about mechanism, why cystic change occurs in metastasizing dermatofibroma. They supposed that cyst formation probably occurred for chronic involvement of alveolar parenchyma. Although prognosis was favorable after complete cutaneous dermatofibroma excision, a regular follow-up with chest x-ray may be needed after excision of a cellular dermatofibroma because the tumors can metastasize to the lung without previous local recurrence or lymph node metastasis [3,7]. We experienced very rare case of metastasizing dermatofibroma in lung. When there is unusual pattern of cystic
67
lung lesions, dermatofibroma metastasis should be considered once, at least, if the patients had history of dermatofibroma of the skin. References [1] Colome-Grimmer MI, Evans HL. Metastasizing cellular dermatofibroma. A report of two cases. Am J Surg Pathol 1994;20:1361 - 7. [2] Joseph MG, Colby TV, Swensen SJ, et al. Multiple cystic fibrohistiocytic tumors of the lung: report of two cases. Mayo Clin Proc 1990; 665:192 - 7. [3] Calonje E, Mentzel T, Fletcher CDM. Cellular benign fibrous histiocytoma. Am J Surg Pathol 1994;18:668 - 76. [4] Osborn M, Mandys V, Beddow E, et al. Cystic fibrohistiocytic tumours presenting in the lung: primary or metastatic disease? Histopathology 2003;43:556 - 62. [5] Colby TV. Metastasizing dermatofibroma. Am J Surg Pathol 1997; 21:976. [6] Gillou L, Gebhard S, Salmeron M, et al. Metastasizing fibrous histiocytoma of the skin: a clinicopathologic and immunohistochemical analysis of the three cases. Mod Pathol 2000;13:654 - 60. [7] De Hertogh G, Bergmans G, Molderz C, et al. Cutaneous cellular fibrous histiocytoma metastasizing to the lungs. Histopathology 2002; 41:85 - 6.
Metastasizing dermatofibroma in lung MiJin Gu, MD, MSa,4, KyungRak Sohn, MD, PhDa, DongJa Kim, MD, PhDa, ByungHo Kim, MD, PhDb b
a Department of Pathology, Daegu Fatima Hospital, Daegu 701-600, Korea Department of Thoracic Surgery, Daegu Fatima Hospital, Daegu 701-600, Korea
Abstract
Benign dermatofibroma is very common skin tumor and can very rarely metastasize. We report a case of metastasizing dermatofibroma on a 36-year-old woman who presented multiple bilateral lung nodules. She underwent incisional biopsy for cellular dermatofibroma of the right shoulder 7 years ago. Chest computed tomographic scanning shows multiple nodules in both lung fields. Segmental and wedge resections were done. Grossly, the masses were hemorrhagic cysts. Microscopically, there were dilated cystic airspaces. The airspaces were lined by respiratory and metaplastic squamous epithelium with underlying layers of fibrohistiocytic spindle cells with storiform and fascicular pattern. The tumor cells stained for CD68 and CD10. The lung mass shows same histologic features with skin lesion. D 2007 Elsevier Inc. All rights reserved.
Keywords:
Dermatofibroma; Lung; Cyst; Metastasis
1. Introduction Benign dermatofibroma is a very common skin tumor and may recur. However, metastasis of dermatofibroma is a very uncommon event [1- 3]. To the best of our knowledge, 12 cases were reported in the English literature since the first description by Joseph et al [2] in 1990. On the observations of previous published cases, the tumors occur in young male adults and present as multiple cavitary or cystic lesions (Table 1). We report a rare case of metastasizing dermatofibroma in a 36-year-old woman without respiratory symptom and review of literatures. 2. Case report A 36-year-old woman visited our hospital for mental change and deviation of tongue. She had a history of diabetes mellitus, hypertension, and hyperthyroidism. At the time of initial examination, she had no respiratory symptom. She had excised cutaneous shoulder nodule 7 years ago. The mass was a cellular dermatofibroma. We could not review the previous skin slide because the slides and blocks were 4 Corresponding author. Tel.: +82 53 940 7272; fax: +82 53 940 7273. E-mail address: [email protected] (M.J. Gu). 1092-9134/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.anndiagpath.2005.12.001
scrapped. Chest computed tomographic (CT) scanning shows multiple discrete, margined round nodules in both lung fields (Fig. 1). Wedge and segmental resections were done. Grossly, hemorrhagic cystic masses were noted: 3 in the right upper lobe, 1 in the right middle lobe, 2 in the right lower lobe, 5 in the left upper lobe, and 4 in the left lower lobe of lung. The size of cysts was 1.2 to 3.9 cm. They were multilocular cysts containing chocolate-colored hemorrhagic material, similar to endometriosis of ovary (Fig. 2). Microscopically, there were dilated cystic airspaces. The airspaces were lined by respiratory epithelium with underlying layers of tumor cells. The tumor cells were composed of plump to spindled fibrohistiocytic cells arranged in storiform growth pattern (Fig. 3). There were no cellular pleomorphism, atypical mitosis, and necrosis. Foamy macrophages, hemosiderin depositions, and cholesterol clefts were observed (Fig. 4). On immunohistochemistry, the tumor cells were stained for CD68 (514H12, Predilution; Ventana, Tucson, AZ) and CD10 (56C6, Predilution; Ventana), but negative on CD34 (QBEnd/10, Predilution; Ventana), CD117 (Polyclonal, 1:50; Dako, Carpinteria, CA) and estrogen receptor (6F11, Predilution; Ventana) was noted (Fig. 5). After the operation of lung, we examined the previous skin lesion and found small remaining cellular dermatofibroma due to previous incomplete excision. The
M.J. Gu et al. / Annals of Diagnostic Pathology 11 (2007) 64 – 67
65
Table 1 Clinical findings of metastatic dermatofibroma in lung Case
Sex
Age
Respiratory symptom
Radiologic or pathological finding
Previous cutaneous lesion
Follow-up (y)
1 2 3 4
M M M M
38 54 35 29
Bilateral Bilateral Bilateral Multiple
Not identified CHF (excluded 23 y ago) Not identified CHF (10 y ago on back)
Alive with disease at 2 y Alive with disease at 2 y Alive with disease at 2 y Not known
5 6
M M
65 30
Bilateral multiple nodular opacities Bilateral nodular opacities
Not identified Recurrent DH (5 y ago on back)
Alive with disease at 2 y Alive with disease at 2 y
7 8 9 10 11 12 13
M F M M M M F
25 30 19 40 33 33 36
Hemoptysis Hemoptysis Not known Pneumonia, pneumothorax Asymptomatic Dyspnea, pneumothorax Hemoptysis Pneumothorax Not known Not known Fatigue No No
Bilateral diffuse cavities Bilateral cystic lesions Cystic lesion on gross finding Cystic lesion on gross finding Multiple masses, not cystic Bilateral multiple cystic lesions Bilateral multiple cystic lesions
Not identified DFP (17 y ago) CHF (1.5 y ago) CHF (7 y ago) CFH (2 y ago) CFH (9 y ago) CFH (7 y ago)
Alive with disease Not known Alive with disease Alive with disease Alive with disease Alive with disease Alive with disease
many nodular and cavitating opacity with many nodules cystic lung nodules bilateral cavitary lesions
at 2 y at 2 y at 2 y at 6 y at 1 y
M, male; F, female; CFH, Cellular fibrous histiocytoma; DF, Dermatofibroma; DFP, Dermatofibrosarcoma protuberans.
mass was 0.4 0.3 cm in size and showed the same histologic features with lung lesion (Fig. 6).
sarcoma [1,6]. We had difficulty of diagnosis on initial small wedge resection because the tumor cells showed positive for CD10 and negative for CD68. And small uterine mass was
3. Discussion Metastasis of dermatofibroma is a very uncommon event [1- 3]. Most previously reported cases were metastasis of cellular dermatofibroma [4,5]. The tumors often occur in young male adults, and they had excision cellular dermatofibroma of skin 1.5 to 23 years ago before lung disease [4]. Gillou et al [6] described that relatively large size, high cellularity, aneurysmal changes, marked cellular pleomorphism, high mitotic figures, tumor necrosis, and local recurrence are risk factors for metastasis. Differential diagnosis includes lymphangioleiomyomatosis, dermatofibroma, angiomatoid fibrous histiocytoma, dermatofibrosarcoma protuberans, solitary fibrous tumor in lung, endometriosis, and metastatic endometrial stromal
Fig. 1. Well-margined multiple masses present in right lung on chest CT scanning.
Fig. 2. Grossly, the lung masses are hemorrhagic cysts (from right: upper, middle, and lower lobe mass).
Fig. 3. Sections of the lung show the wall of cyst is lined by respiratory epithelium with underlying layers of spindle cells.
66
M.J. Gu et al. / Annals of Diagnostic Pathology 11 (2007) 64 – 67
Fig. 4. The tumor cells composed of fibrohistiocytic spindle cells with storiform and fascicular pattern. Foam cells, inflammatory cells, and hemosiderin depositions are mixed.
detected in abdominal sonogram. However, on whole masses excision, the tumor cells were positive on CD68 and CD10, and the uterine mass was a leiomyoma. Lymphangioleiomyomatosis should be excluded because it
Fig. 6. Sections of skin show remaining dermatofibroma in reticular dermis.
occurs women of childbearing age. However, the spindle tumor cells of lymphangioleiomyomatosis were positive on smooth muscle actin and HMB-45 [6]. Angiomatoid fibrous histiocytoma can be excluded in our case because the tumors had storiform pattern and lacked peripheral cuffing of lymphocytes and plasma cells (a fibrous pseudocapsule)
Fig. 5. On immunohistochemical staining, the tumor cells stain for CD68 and CD10. The lining epithelial cells stain for thyroid transcription factor (TTF)-1.
M.J. Gu et al. / Annals of Diagnostic Pathology 11 (2007) 64 – 67
[1,6]. Dermatofibrosarcoma was much less dermarcated and showed more uniform spindle tumor cells, and the tumor cells were positive for CD34 [6]. Osborn et al [4] reported a review of 10 published cases of metastasizing dermatofibroma. The masses were presented as cystic fibrohistiocytic tumor in the lung. Our case also showed multiple hemorrhagic cysts as endometriosis of ovary. The masses were presented with multiple bilateral cystic lung lesions or nodular cavitary lesions on imaging study (Table 1). It is uncertain, about mechanism, why cystic change occurs in metastasizing dermatofibroma. They supposed that cyst formation probably occurred for chronic involvement of alveolar parenchyma. Although prognosis was favorable after complete cutaneous dermatofibroma excision, a regular follow-up with chest x-ray may be needed after excision of a cellular dermatofibroma because the tumors can metastasize to the lung without previous local recurrence or lymph node metastasis [3,7]. We experienced very rare case of metastasizing dermatofibroma in lung. When there is unusual pattern of cystic
67
lung lesions, dermatofibroma metastasis should be considered once, at least, if the patients had history of dermatofibroma of the skin. References [1] Colome-Grimmer MI, Evans HL. Metastasizing cellular dermatofibroma. A report of two cases. Am J Surg Pathol 1994;20:1361 - 7. [2] Joseph MG, Colby TV, Swensen SJ, et al. Multiple cystic fibrohistiocytic tumors of the lung: report of two cases. Mayo Clin Proc 1990; 665:192 - 7. [3] Calonje E, Mentzel T, Fletcher CDM. Cellular benign fibrous histiocytoma. Am J Surg Pathol 1994;18:668 - 76. [4] Osborn M, Mandys V, Beddow E, et al. Cystic fibrohistiocytic tumours presenting in the lung: primary or metastatic disease? Histopathology 2003;43:556 - 62. [5] Colby TV. Metastasizing dermatofibroma. Am J Surg Pathol 1997; 21:976. [6] Gillou L, Gebhard S, Salmeron M, et al. Metastasizing fibrous histiocytoma of the skin: a clinicopathologic and immunohistochemical analysis of the three cases. Mod Pathol 2000;13:654 - 60. [7] De Hertogh G, Bergmans G, Molderz C, et al. Cutaneous cellular fibrous histiocytoma metastasizing to the lungs. Histopathology 2002; 41:85 - 6.