- Email: [email protected]
Epidermotropic eccrine porocarcinoma
III
I1|1
Epidermotropic eccrine porocarcinoma Nerea G. Landa, MD, and R. K. Winkelmann, MD, PhD Rochester, Minnesota Three cases of epidermotropic eccrine porocarcinoma are summarized and compared with reported cases. All patients had a long-standing tumor on a lower extremity that rapidly metastasized to the skin and proximal lymph nodes. The histologic picture was consistent with an intraepidermal eccrine sweat gland carcinoma. (J AM AcAI) DERMATOL1991 ;24:27-31.)
O f the many patterns of presentation of sweat gland carcinoma, neoplastic proliferations of the intraepidermal eccrine sweat duct are among the rarest. In 1963 Pinkus and Mehregan I described a sweat gland carcinoma of the lower extremity with later involvement of the thigh. They called it epidermotropic eccrine carcinoma on the basis of the tendency of the cells to proliferate and spread intraepidermally in a pagetoid pattern. Isolated cases of epidermotropic eccrine carcinoma had been reported until Mehregan et al. 2 reported 18 cases and emphasized that this tumor was most probably epidermotropic porocarcinoma. We report three new cases of this tumor to emphasize the pattern of clinical presentation.
!
CASE REPORTS
Case 1 In February 1989 a 78-year-old man had a wide excision of a 3 cm tumor on his left buttock that had been present for several years and had recently increased in size. A diagnosis of epidermotropic eccrine carcinoma was made. In March 1989 he had the sudden onset of painful swelling of the left leg (Fig. 1). A diagnosis of deep venous thrombosis was made. At that time a computed tomographic (CT) scan of the abdomen did not show any abnormality. In May 1989 he was hospitalized; bed rest with leg elevation was prescribed, which resulted in prompt response of the edema. A CT scan showed slight left inguinal adenopathy and possible thrombosis in the left common femoral vein. In November 1989 three red papules were visible in the scar of the incision on his left buttock. They were widely
From the Department of Dermatology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota. Accepted for publication June 4, 1990. No reprints available. 16/'1/'22995
27
Fig. 1. Case 1. Lymphedema of left lower leg is shown. Metastases at top have been previously excised.
excised. A biopsy specimen again showed epidermotropic eccrine carcinoma. The CT scan showed a 2 cm infiltrative left paraaortic mass; cytologic examination was positive for carcinoma cells.
28
Journal of the American Academy of Dermatology
Landa and Winkelmann
Fig. 2. Case 2. Metastatic lesions on flank in zosteriform distribution.
Fig. 3, Case 3. Widespread metastasis on left thigh on lymphedematous extremity.
Case 2
A 75-year-old woman was hospitalized in July 1973 for swelling of the left leg and skin lesions on the left flank. She first noted a lump in her left groin in May 1972 that was excised in July 1972 and diagnosed as "adenocarcinoma present in lymph nodes." A iymphangiogram showed "blockage." After operation, she experienced progressive painful swelling of the left leg. In November 1972 she received radiotherapy for the left leg and groin, with no improvement. In July 1973 several nodules and a burning sensation developed in a band on her left flank. Physical examination disclosed several erythematous verrueous nodules at the T12 level in a zosteriform pattern (Fig. 2). A biopsy specimen led to a diagnosis of "grade
4 sweat gland carcinoma." Radiographs disclosed an infiltrative process in the right middle and lower lobes, suggestive of metastases, and a malignant destruction of the L1 pedicle. She was discharged in August 1973 and is known to have had undetermined chemotherapy in September 1973. She died in November 1973. No death report was available, and no autopsy was performed. Case 3
A 24-year-old woman was hospitalized in June 1970 for pain and swelling of the left leg with skin lesions. At 14 years of age she had first noticed a small lump on the medial aspect of her left shin; the lump was fulgurated. When the patient was 15 years old, there was a local re-
Volume 24 Number 1 January 1991
Epidermotropic eccrine porocarcinoma 29
Fig. 4. Case 4. Well-demarcated nest of tumor ceils in acanthotie epidermis. Dermal invasion is seen. Scattered ductal lumina are evident in tumor nests. (Hematoxylin-eosin stain; x55.)
currence that was excised, diagnosed as "trichoepithelioma," and treated with wide excision. Again when the patient was 16 years old, there was a local recurrence treated with a wide excision and graft. Another recurrence when the patient was 20 years old was treated again by excision and grafting; histologic results raised the question of sweat gland carcinoma. At 23 years of age she noticed a lump in her left groin; the lump was a tumor metastasis and radical left femoral deep and superficial node dissection was done. In January 1970 a paraaortic node dissection was done. Tumor was present throughout in many nodes. After surgery, cobalt radiotherapy was given. The edema progressed to involve her groin and genital area. In March 1970 cobalt radiotherapy to the inverted-Y field was given. In May 1970 the edema involved the abdominal wall, and she had anemia, leukopenia, weight loss, and pain. On admission in June 1970, physical examination disdosed superficial nodularity along the left thigh, with an infiltrated skin eruption (Fig. 3). A biopsy specimen showed epidermotropic eccrine carcinoma. Chest radiography showed a diffuse interstitial process in both lungs, not conclusive for metastases. She died in July 1973 of generalized metastatic tumor. No autopsy was performed. HISTOPATHOLOGIC FEATURES The tumor masses are clearly demarcated and frequently rounded nests of polygonal clear cells are seen (Fig. 4). The cytoplasm may be slightly acidophilic. The nuclei are large, and hyperchromatic mitoses are present. The masses characteristically infiltrate the epidermis and may have isolated nests
Fig. 5. Clear cell intraepidermal cell masses. (Hematoxylin-eosin stain; • of cells within the epidermis, or isolated individual cells may be observed (Fig. 5). The epidermis frequently is acanthotic, although in some areas at-
30
Journal of the American Academy of Dermatology
Landa and Winkelrnann
Table I. Epidermotropic eccrine porocarcinoma
Author(s) Pinkus and Mehregan ~ Miura 3 Krinitz 4 Grosshans et al. 5 Gschnait et al. 6 Turner et al. 7 Shaw et al. s Mehregan et al. 2 Roach 9 Bottles et al. 1~ Claudy et al.~ l Ryan et al) 2 D6ring and Seifert 13 Present cases
Outcome
Loca~.~_~LLymphedema I involvem~t 82 68 77 84 69 63 85 61 58 63 61 60 81 56 76 87 78 75 24 78
F M F F ? M F F F M M M F M M M M F F M
L foot R calf Lower leg L foot R foot L umbilicus Loin Foot Buttock Foot R thigh R thigh R calf L buttock R thigh L flank Back L groin Lshin Lbuttoek
4 12 Years 11/2 50 19 ? 40 6 Years 2 2 Weeks 5 2 Years 3 1~ 10 Years
+ + + + + + ? ? ? + + + + + + +
+ + + + + + + + + + + + + + + + + + + +
+ + + + + + + ? ? + + + + + + + + + +
? + + + ? ? ? ? + ? + ? -
Dead Dead Dead Dead ? ? ? ? ? Dead ? Dead ? Dead Dead Dead Dead Dead Alive with metastases
+, Present; - , absent; 2, unknown.
rophy may be observed. Hyperkeratinization and parakeratosis are usually present in areas that overlie the invasion of the epidermis. Masses of tumor often invade the papillary dermis and are found abutting the proliferative epidermis. Often the entire dermis may be involved. On occasion, invasion of the dermal lymphatics is observed, or the nests of cells may be isolated and free in the dermis. The tumor cells may be intensely basophilic or they may show clear cell differentiation. Because of their size and regularity, they can represent epidermoid carcinoma. The presence of lumina between the cells is one indication of their adenocarcinomatous origin. Similarly, individual cells m a y show dilated, rounded spaces within the cytoplasm. The tumor cells are attached by intercellular bridges but are not connected to surrounding epidermal or dermal cells. The dermis is generally replaced by the tumor masses. This is not complete because some desmoplastic stromal reaction in the dermis can be seen, together with new blood vessel formation. Inflammatory reaction varies according to the amount of tissue destruction. Inflammatory round cells may be seen within the tumor masses. Periodic acid-Schiff (PAS)-positive, diastase-
labile deposits of glycogen are found in the cytoplasm of the tumor cells of our three cases. Acid mucopolysaccharide was demonstrated by alcian blue stain in the connective tissue between the tumor nests. Carcinoembryonic antigen (CEA) was found lining ductal lumina and some periductal cells of the tumor mass in the three cases. Cytokeratin was demonstrated in the cytoplasm of cases 1 and 2 in some rare localized areas. Tests for S-100 protein were negative in all cases. The metastatic lesions in the lymph nodes and in the skin are also composed of rounded, well-demarcated masses or nests of polygonal clear cells. These nests are identical, in general, to the histopathologic features of the primary lesion. The lymph node parenchyma is destroyed, and the entire mass of the lymph node may be involved by contiguous tumor masses. Peripheral lymphatics at the edge of the lymph node may show invasion of the tumor nests.
DISCUSSION We believe that Pink-us and Mehregan l described a unique clinical presentation of solid edema of the thigh, groin, buttock, or abdomen associated with tumor papules and nodules that demonstrated intraepidermal and juxtaepidermal masses of sweat gland carcinoma.
Volume 24 Number 1 January 1991
The clinical presentation may occur after a noduloverrucous tumor of the distal lower extremities that changes suddenly and metastasizes, as in the original Pinkus and Mehregan case and in our case 3. The origin of the tumor may be in the proximal tissue of the lower trunk, the area that later develops the solid edema/metastasis clinical complex, as in our cases 1 and 2. A recurring feature is the presence of epidermal papules of metastatic and epidermotropic carcinoma. This was present in our cases and in most later cases in the literature t-13 (Table I). The cases share the same clinical features with Pinkus and Mehregan's case and our cases. It is our belief that all will eventually behave in the same way. The common histopathologic pattern is the intraepidermal proliferation of neoplastic cells, mostly as nests but also as individual cells. They tend to invade the underlying dermis and ulcerate the epidermis. The cells are polygonal with prominent nuclei and a considerable amount of mitosis. They can be intensely basophilic or clear and attach to each other by intracellular bridges. Inside the tumor mass, many ductal lumina can be identified, l"s, m-13 Glycogen was present in the cytoplasm of the tumor cells in our three cases in support of their eecrine nature) 4 Staining for C E A was positive in the ductal lumina and periluminal cells in the tumor, the same as in most sweat gland-derived tumors. 15 The presence of keratin in some areas in two of our cases was interpreted as a sign of keratinization of intraepidermal duct ceUs. 14 Although S-100 protein has been found in eccrine gland carcinoma in isolated cases, 16 our three cases were negative. Porocarcinoma does not always show these clinical features. Many porocarcinomas are epidermal or dermal tumors that do not metastasize and are completely excised without recurrence. 17,18 Similarity of histopathologic features should cause consideration of metastatic adenocarcinoma to the skin. The prognosis of epidermotropic eccrine carcinoma is poor and most patients whose cases have been described in the literature died rapidly (Table 1). Efforts must be made to establish the absence of other carcinomas. Despite the typical histologic features and pattern of development, confusion with carcinoma of the prostate and bladder, cloacogenic carcinoma, and extramammary Paget's disease is possible..Our patient (case 1) had had carcinoma of the prostate, and the circulating prostate antigen level was slightly increased. However, the absence of bone metastasis or recurrence in the prostatic bed
Epidermotropic eccrine porocarcinoma
31
and the characteristic clinical and pathologic findings differentiated this case from visceral cancer. Extramammary Paget's cases usually have mucincontaining cells; porocarcinoma has acid muc0polysaccharide only in the stroma. S-100 proteinnegative and silver-negative tissue and the glandular appearance of the tissue rule out malignant melanoma. Early recognition of this tumor allows the only \ chance at definitive treatment b y surgical excision. Delay ensures progressive carcinomatosis and death.
REFERENCES 1. Pink-usH, Mehregan AH. Epidermotropic eccrine carcinoma: a case combining features of eccrine poroma and Paget's dermatosis. Arch Dermatol 1963;88:597-604. 2. Mehregan AH, Hashimoto K, Rahbari H. Eccrine adenocarcinoma: a clinicopathologic study of 35 cases. Arch Dermatol 1983;119:104-14. 3. Miura Y. Epidermotropic eccrine carcinoma. Jpn J Dermatol (Series B) 1968;78:226-30. 4. Krinitz K. Malignes intraepidermales ekkrines porom. Z Haut Geschtechtskr 1972;47:9-17. 5. Grosshans E, Vetter JM, Capesius C. Poromes eccrines malins (poro-6pith61iomas, porocarcinomes). Ann Anat Pathol 1975;20:381-94. 6. Gschnait F, Horn F, Lindlbauer K, et al. Eccrine porocarcinoma. J Cutan Pathol 1980;7:349-53. 7. Turner JJ, MaxwellL, BursleGA. Eccrineporocarcinoma: a case report with light microscopyand ultrastructure. Pathology 1982;14:469-75. 8. Shaw M, McKee PH, Lowe D, et al. Malignant eccrine poroma: a study of twenty-seven cases. Br J Dermatol 1982;107:675-80. 9. Roach MiII. A malignant eccrine poroma responds to isotretinoin (13-cis-retinoicacid). Ann Intern Med 1983; 99:486-8. 10. BottlesK, SagebielRW, McNutt NS, et al. Malignant eccrlne poroma:casereport and reviewof the literature. Cancer 1984;53:1579-85. 11. Claudy AL, Garcier F, Kanitakis J. Eccrine porocarcinoma: ultrastructural and immunological study. J Dermatol (Tokyo) 1984;11:282-6. 12. Ryan J F, Darley CR, PollockD J. Malignant eccrine poroma: report of three cases. J Clin Pathol 1986;39:1099-104. 13. D~Sring HF, Seifert HW. Ekkrines Porokarzinom (malignes ekkrines Porom)---eine .Verlaufsbeobachtung. Z Hautkr 1987;62:1051-2, 1055. 14. HashimotoK, Gross BG, Lever WF. The ultrastructure of the skin of human embryos. I. The intraepidermal eccrine sweat duct. J Invest Dermatol 1965;45:139-51. 15. Penneys NS, Nadji M, Ziegels-Weissman J, et al. Careinoembryonic antigen in sweat-gland carcinomas. Cancer 1982;50:1608-11. 16. Mita H, Takigawa M, Iwatsuki K, et al. An immunohistologic study on eccrine gland carcinoma. J AM ACADDI~RMATOL1989;20:693-6. 17. RuffieuxC, Ramelet A-A. Porocarcinomeeccrine: pr6sentation de 4 cas. Dermatologica 1985;170:202-6. 18. Puttick L, Ince P, Comaish JS. Three cases of ecerine poroearcinoma. Br J Dermatol 1986;115:111-6.
I1|1
Epidermotropic eccrine porocarcinoma Nerea G. Landa, MD, and R. K. Winkelmann, MD, PhD Rochester, Minnesota Three cases of epidermotropic eccrine porocarcinoma are summarized and compared with reported cases. All patients had a long-standing tumor on a lower extremity that rapidly metastasized to the skin and proximal lymph nodes. The histologic picture was consistent with an intraepidermal eccrine sweat gland carcinoma. (J AM AcAI) DERMATOL1991 ;24:27-31.)
O f the many patterns of presentation of sweat gland carcinoma, neoplastic proliferations of the intraepidermal eccrine sweat duct are among the rarest. In 1963 Pinkus and Mehregan I described a sweat gland carcinoma of the lower extremity with later involvement of the thigh. They called it epidermotropic eccrine carcinoma on the basis of the tendency of the cells to proliferate and spread intraepidermally in a pagetoid pattern. Isolated cases of epidermotropic eccrine carcinoma had been reported until Mehregan et al. 2 reported 18 cases and emphasized that this tumor was most probably epidermotropic porocarcinoma. We report three new cases of this tumor to emphasize the pattern of clinical presentation.
!
CASE REPORTS
Case 1 In February 1989 a 78-year-old man had a wide excision of a 3 cm tumor on his left buttock that had been present for several years and had recently increased in size. A diagnosis of epidermotropic eccrine carcinoma was made. In March 1989 he had the sudden onset of painful swelling of the left leg (Fig. 1). A diagnosis of deep venous thrombosis was made. At that time a computed tomographic (CT) scan of the abdomen did not show any abnormality. In May 1989 he was hospitalized; bed rest with leg elevation was prescribed, which resulted in prompt response of the edema. A CT scan showed slight left inguinal adenopathy and possible thrombosis in the left common femoral vein. In November 1989 three red papules were visible in the scar of the incision on his left buttock. They were widely
From the Department of Dermatology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota. Accepted for publication June 4, 1990. No reprints available. 16/'1/'22995
27
Fig. 1. Case 1. Lymphedema of left lower leg is shown. Metastases at top have been previously excised.
excised. A biopsy specimen again showed epidermotropic eccrine carcinoma. The CT scan showed a 2 cm infiltrative left paraaortic mass; cytologic examination was positive for carcinoma cells.
28
Journal of the American Academy of Dermatology
Landa and Winkelmann
Fig. 2. Case 2. Metastatic lesions on flank in zosteriform distribution.
Fig. 3, Case 3. Widespread metastasis on left thigh on lymphedematous extremity.
Case 2
A 75-year-old woman was hospitalized in July 1973 for swelling of the left leg and skin lesions on the left flank. She first noted a lump in her left groin in May 1972 that was excised in July 1972 and diagnosed as "adenocarcinoma present in lymph nodes." A iymphangiogram showed "blockage." After operation, she experienced progressive painful swelling of the left leg. In November 1972 she received radiotherapy for the left leg and groin, with no improvement. In July 1973 several nodules and a burning sensation developed in a band on her left flank. Physical examination disclosed several erythematous verrueous nodules at the T12 level in a zosteriform pattern (Fig. 2). A biopsy specimen led to a diagnosis of "grade
4 sweat gland carcinoma." Radiographs disclosed an infiltrative process in the right middle and lower lobes, suggestive of metastases, and a malignant destruction of the L1 pedicle. She was discharged in August 1973 and is known to have had undetermined chemotherapy in September 1973. She died in November 1973. No death report was available, and no autopsy was performed. Case 3
A 24-year-old woman was hospitalized in June 1970 for pain and swelling of the left leg with skin lesions. At 14 years of age she had first noticed a small lump on the medial aspect of her left shin; the lump was fulgurated. When the patient was 15 years old, there was a local re-
Volume 24 Number 1 January 1991
Epidermotropic eccrine porocarcinoma 29
Fig. 4. Case 4. Well-demarcated nest of tumor ceils in acanthotie epidermis. Dermal invasion is seen. Scattered ductal lumina are evident in tumor nests. (Hematoxylin-eosin stain; x55.)
currence that was excised, diagnosed as "trichoepithelioma," and treated with wide excision. Again when the patient was 16 years old, there was a local recurrence treated with a wide excision and graft. Another recurrence when the patient was 20 years old was treated again by excision and grafting; histologic results raised the question of sweat gland carcinoma. At 23 years of age she noticed a lump in her left groin; the lump was a tumor metastasis and radical left femoral deep and superficial node dissection was done. In January 1970 a paraaortic node dissection was done. Tumor was present throughout in many nodes. After surgery, cobalt radiotherapy was given. The edema progressed to involve her groin and genital area. In March 1970 cobalt radiotherapy to the inverted-Y field was given. In May 1970 the edema involved the abdominal wall, and she had anemia, leukopenia, weight loss, and pain. On admission in June 1970, physical examination disdosed superficial nodularity along the left thigh, with an infiltrated skin eruption (Fig. 3). A biopsy specimen showed epidermotropic eccrine carcinoma. Chest radiography showed a diffuse interstitial process in both lungs, not conclusive for metastases. She died in July 1973 of generalized metastatic tumor. No autopsy was performed. HISTOPATHOLOGIC FEATURES The tumor masses are clearly demarcated and frequently rounded nests of polygonal clear cells are seen (Fig. 4). The cytoplasm may be slightly acidophilic. The nuclei are large, and hyperchromatic mitoses are present. The masses characteristically infiltrate the epidermis and may have isolated nests
Fig. 5. Clear cell intraepidermal cell masses. (Hematoxylin-eosin stain; • of cells within the epidermis, or isolated individual cells may be observed (Fig. 5). The epidermis frequently is acanthotic, although in some areas at-
30
Journal of the American Academy of Dermatology
Landa and Winkelrnann
Table I. Epidermotropic eccrine porocarcinoma
Author(s) Pinkus and Mehregan ~ Miura 3 Krinitz 4 Grosshans et al. 5 Gschnait et al. 6 Turner et al. 7 Shaw et al. s Mehregan et al. 2 Roach 9 Bottles et al. 1~ Claudy et al.~ l Ryan et al) 2 D6ring and Seifert 13 Present cases
Outcome
Loca~.~_~LLymphedema I involvem~t 82 68 77 84 69 63 85 61 58 63 61 60 81 56 76 87 78 75 24 78
F M F F ? M F F F M M M F M M M M F F M
L foot R calf Lower leg L foot R foot L umbilicus Loin Foot Buttock Foot R thigh R thigh R calf L buttock R thigh L flank Back L groin Lshin Lbuttoek
4 12 Years 11/2 50 19 ? 40 6 Years 2 2 Weeks 5 2 Years 3 1~ 10 Years
+ + + + + + ? ? ? + + + + + + +
+ + + + + + + + + + + + + + + + + + + +
+ + + + + + + ? ? + + + + + + + + + +
? + + + ? ? ? ? + ? + ? -
Dead Dead Dead Dead ? ? ? ? ? Dead ? Dead ? Dead Dead Dead Dead Dead Alive with metastases
+, Present; - , absent; 2, unknown.
rophy may be observed. Hyperkeratinization and parakeratosis are usually present in areas that overlie the invasion of the epidermis. Masses of tumor often invade the papillary dermis and are found abutting the proliferative epidermis. Often the entire dermis may be involved. On occasion, invasion of the dermal lymphatics is observed, or the nests of cells may be isolated and free in the dermis. The tumor cells may be intensely basophilic or they may show clear cell differentiation. Because of their size and regularity, they can represent epidermoid carcinoma. The presence of lumina between the cells is one indication of their adenocarcinomatous origin. Similarly, individual cells m a y show dilated, rounded spaces within the cytoplasm. The tumor cells are attached by intercellular bridges but are not connected to surrounding epidermal or dermal cells. The dermis is generally replaced by the tumor masses. This is not complete because some desmoplastic stromal reaction in the dermis can be seen, together with new blood vessel formation. Inflammatory reaction varies according to the amount of tissue destruction. Inflammatory round cells may be seen within the tumor masses. Periodic acid-Schiff (PAS)-positive, diastase-
labile deposits of glycogen are found in the cytoplasm of the tumor cells of our three cases. Acid mucopolysaccharide was demonstrated by alcian blue stain in the connective tissue between the tumor nests. Carcinoembryonic antigen (CEA) was found lining ductal lumina and some periductal cells of the tumor mass in the three cases. Cytokeratin was demonstrated in the cytoplasm of cases 1 and 2 in some rare localized areas. Tests for S-100 protein were negative in all cases. The metastatic lesions in the lymph nodes and in the skin are also composed of rounded, well-demarcated masses or nests of polygonal clear cells. These nests are identical, in general, to the histopathologic features of the primary lesion. The lymph node parenchyma is destroyed, and the entire mass of the lymph node may be involved by contiguous tumor masses. Peripheral lymphatics at the edge of the lymph node may show invasion of the tumor nests.
DISCUSSION We believe that Pink-us and Mehregan l described a unique clinical presentation of solid edema of the thigh, groin, buttock, or abdomen associated with tumor papules and nodules that demonstrated intraepidermal and juxtaepidermal masses of sweat gland carcinoma.
Volume 24 Number 1 January 1991
The clinical presentation may occur after a noduloverrucous tumor of the distal lower extremities that changes suddenly and metastasizes, as in the original Pinkus and Mehregan case and in our case 3. The origin of the tumor may be in the proximal tissue of the lower trunk, the area that later develops the solid edema/metastasis clinical complex, as in our cases 1 and 2. A recurring feature is the presence of epidermal papules of metastatic and epidermotropic carcinoma. This was present in our cases and in most later cases in the literature t-13 (Table I). The cases share the same clinical features with Pinkus and Mehregan's case and our cases. It is our belief that all will eventually behave in the same way. The common histopathologic pattern is the intraepidermal proliferation of neoplastic cells, mostly as nests but also as individual cells. They tend to invade the underlying dermis and ulcerate the epidermis. The cells are polygonal with prominent nuclei and a considerable amount of mitosis. They can be intensely basophilic or clear and attach to each other by intracellular bridges. Inside the tumor mass, many ductal lumina can be identified, l"s, m-13 Glycogen was present in the cytoplasm of the tumor cells in our three cases in support of their eecrine nature) 4 Staining for C E A was positive in the ductal lumina and periluminal cells in the tumor, the same as in most sweat gland-derived tumors. 15 The presence of keratin in some areas in two of our cases was interpreted as a sign of keratinization of intraepidermal duct ceUs. 14 Although S-100 protein has been found in eccrine gland carcinoma in isolated cases, 16 our three cases were negative. Porocarcinoma does not always show these clinical features. Many porocarcinomas are epidermal or dermal tumors that do not metastasize and are completely excised without recurrence. 17,18 Similarity of histopathologic features should cause consideration of metastatic adenocarcinoma to the skin. The prognosis of epidermotropic eccrine carcinoma is poor and most patients whose cases have been described in the literature died rapidly (Table 1). Efforts must be made to establish the absence of other carcinomas. Despite the typical histologic features and pattern of development, confusion with carcinoma of the prostate and bladder, cloacogenic carcinoma, and extramammary Paget's disease is possible..Our patient (case 1) had had carcinoma of the prostate, and the circulating prostate antigen level was slightly increased. However, the absence of bone metastasis or recurrence in the prostatic bed
Epidermotropic eccrine porocarcinoma
31
and the characteristic clinical and pathologic findings differentiated this case from visceral cancer. Extramammary Paget's cases usually have mucincontaining cells; porocarcinoma has acid muc0polysaccharide only in the stroma. S-100 proteinnegative and silver-negative tissue and the glandular appearance of the tissue rule out malignant melanoma. Early recognition of this tumor allows the only \ chance at definitive treatment b y surgical excision. Delay ensures progressive carcinomatosis and death.
REFERENCES 1. Pink-usH, Mehregan AH. Epidermotropic eccrine carcinoma: a case combining features of eccrine poroma and Paget's dermatosis. Arch Dermatol 1963;88:597-604. 2. Mehregan AH, Hashimoto K, Rahbari H. Eccrine adenocarcinoma: a clinicopathologic study of 35 cases. Arch Dermatol 1983;119:104-14. 3. Miura Y. Epidermotropic eccrine carcinoma. Jpn J Dermatol (Series B) 1968;78:226-30. 4. Krinitz K. Malignes intraepidermales ekkrines porom. Z Haut Geschtechtskr 1972;47:9-17. 5. Grosshans E, Vetter JM, Capesius C. Poromes eccrines malins (poro-6pith61iomas, porocarcinomes). Ann Anat Pathol 1975;20:381-94. 6. Gschnait F, Horn F, Lindlbauer K, et al. Eccrine porocarcinoma. J Cutan Pathol 1980;7:349-53. 7. Turner JJ, MaxwellL, BursleGA. Eccrineporocarcinoma: a case report with light microscopyand ultrastructure. Pathology 1982;14:469-75. 8. Shaw M, McKee PH, Lowe D, et al. Malignant eccrine poroma: a study of twenty-seven cases. Br J Dermatol 1982;107:675-80. 9. Roach MiII. A malignant eccrine poroma responds to isotretinoin (13-cis-retinoicacid). Ann Intern Med 1983; 99:486-8. 10. BottlesK, SagebielRW, McNutt NS, et al. Malignant eccrlne poroma:casereport and reviewof the literature. Cancer 1984;53:1579-85. 11. Claudy AL, Garcier F, Kanitakis J. Eccrine porocarcinoma: ultrastructural and immunological study. J Dermatol (Tokyo) 1984;11:282-6. 12. Ryan J F, Darley CR, PollockD J. Malignant eccrine poroma: report of three cases. J Clin Pathol 1986;39:1099-104. 13. D~Sring HF, Seifert HW. Ekkrines Porokarzinom (malignes ekkrines Porom)---eine .Verlaufsbeobachtung. Z Hautkr 1987;62:1051-2, 1055. 14. HashimotoK, Gross BG, Lever WF. The ultrastructure of the skin of human embryos. I. The intraepidermal eccrine sweat duct. J Invest Dermatol 1965;45:139-51. 15. Penneys NS, Nadji M, Ziegels-Weissman J, et al. Careinoembryonic antigen in sweat-gland carcinomas. Cancer 1982;50:1608-11. 16. Mita H, Takigawa M, Iwatsuki K, et al. An immunohistologic study on eccrine gland carcinoma. J AM ACADDI~RMATOL1989;20:693-6. 17. RuffieuxC, Ramelet A-A. Porocarcinomeeccrine: pr6sentation de 4 cas. Dermatologica 1985;170:202-6. 18. Puttick L, Ince P, Comaish JS. Three cases of ecerine poroearcinoma. Br J Dermatol 1986;115:111-6.