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Alopecia neoplastica without alopecia: A unique presentation of breast carcinoma scalp metastasis
Journal of the American Academy of Dermatology Volume 31, Number 2, Part 2
3. Kishimoto TK, Springer T A. Human leukocyte adhesion deficiency:molecular basis for a defective immune response to infections of the skin. Curr Probl DermatoI1989;18:10615. 4. O'Gorman MRG, McNally AC, Anderson DC, et al. A rapid whole blood lysis technique for the diagnosis of moderate or severe leukocyte adhesion deficiency (LAD). Ann NY Acad Sci 1993;677:427-30. 5. Anderson DC, Schmalsteig FC, Finegold MJ, et at. The severe and moderate phenotypes of heritable Mac-I, LFA-I deficiency: their quantitative definition and relation to leukocyte dysfunction and clinical features. J Infect Dis 1985; 152:668-89. 6. Waldrop TC, Anderson DC, Hallmon WW, et aL Periodontal manifestations of the heritable Mac-I, LFA-I, de-
Mallon and Dawber ficiency syndrome: clinical, histopathologic and molecular characteristics. J Periodontol 1987;58:400-16. 7. Fischer A, Descamps-Latascha B, Gerita I, et aL Bone-marrow transplantation for inborn error of phagocytic cells associated with defective adherence, chemotaxis, and oxidative response during opsonized particle phagocytosis. Lancet 1983;2:473-6. 8. Le Diest F, Blanche S, Keable H, et al. Successful HLA nonidentical bone marrow transplantation in three patients with the leukocyte adhesion deficiency. Blood 1989;74:51216. 9. Weening RS, Bredius RG, WolfH, et al. Prenatal diagnostic procedure for leukocyte adhesion deficiency. Prenatal Diagn 1991;11:193-7.
Alopecia neoplastica without alopecia: A unique presentation of breast carcinoma scalp metastasis Eleanor Mallon, MBBS, MRCP, and Rodney P. R. Dawber, MA, MBChB, FRCP
Oxford, United Kingdom The scalp is a relatively common site of cutaneous metastasis. The usual presentation is of single or multiple firm scalp nodules. A well-recognized but rarer presentation is alopecia neoplastica that is seenas singleor multipleareas of cicatricial alopecia.We describea unique presentation of scalp metastasis in a patient with breast carcinoma in whom hair loss was clinically inconspicuous. The metastasis responded to combination chemotherapy. (J AM ACAD DERMATOL
1994;31:319-21.)
Cutaneous metastasis from an underlying breast carcinoma is well recognized; the scalp is a relatively common site, possibly because it provides a vascular, immobile environment, suitable for the development of tumor cells. 1, 2 Scalp metastasis may be the first sign of internal malignancy or it may occur many years after the diagnosis of malignancy. The usual presentation is of single or multiple firm scalp nodules that grow rapidly. Alopecia neop1astica is a rare form of cutaneous metastasis and can simulate other causes of alopecia. It initially appears as single or multiple areas of scarring alopecia. Whether this type of response occurs depends on tumor type, duration, and host response. Histologic study of the From the Department of Dermatology, Churchill Hospital. Reprint requests: Eleanor Mallon, MBBS, MRCP, Department of Dermatology, Churchill Hospital, Oxford, Ox3 7U, United Kingdom. Copyright @ 1994 by the American Academy of Dermatology, Inc.
0190-9622/94 $3.00 + 0
16/4/53735
scalp in alopecia neop1astica demonstrates metastatic carcinoma cells in a dense collagenous stroma, with loss of pilosebaceous units. 3 CASE REPORT
A 71-year-old woman had a left total mastectomy and axillary node dissection for adenocarcinoma in 1977. Histopathologic examination showed a lobular invasive carcinoma, and axillary nodes were free of tumor. The patient received a course of radiotherapy. Lymphedema of the left arm developed 2 years after surgery. The disease was clinicallyquiescent until 1991 when the patient reported back and hip pain. A bone scan confirmed the presence of metastasic deposits in the sternum, lumbar vertebrae, and right neck of femur. Symptomatic improvement wasobtained with localized radiotherapy, and tamoxifen therapy, 20 mg daily, was started. Nine months later an erythematous area that involved one half of the scalp had developed gradually during a 4-week period. Examination showed a 12 em X 10 em "granulomatous" plaque involving the right frontotemporal and parietal scalp (Fig. I). There was an area of
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Journal of the American Academy of Dermatology August 1994
Fig. 1. Metastatic breast carcinoma of scalp. Erythematous plaque is present on right side of scalp; hair loss is inconspicuous.
Fig. 2. Scalp biopsy specimen shows diffuseinfiltrate of small, hyperchromatic carcinomatous cells. Normal hair follicles are present. (Hematoxylin-eosin stain, X25.) crusting in the involvedscalp. No hair loss was apparent clinically, and the hair follicles appeared normal with respect to their size, number, and appearance. A biopsy specimen from the scalp revealed that the dermis was diffusely infiltrated by ribbons and clusters of carcinomatous cells. The infiltrating cells were relatively small, hyperchromatic, and had a high nuclear /cytoplasmic ratio (Fig. 2). In addition, carcinomatous cells dissected between bundles of collagen in files. Normal hair follicles were observed, and there was no associated fibroplasia. The patient received six pulses of combination cytotoxic chemotherapy in 12 weeks; the scalp plaque resolved
after the third pulse. However, total nonscarring alopecia of the anagen effluviumtype developed as a result of the chemotherapy.
DISCUSSION Cutaneous metastasis from an underlying carcinoma is not unusual, but it is rare in comparison with metastases to other organs of the body. The overall incidence of cutaneous metastases is 2.7% 1 to 4.4%. 4 The metastasis may occur by direct extension to the skin, extension through lymphatics, or by accidental implantation during a surgical procedure. In addi-
Journal of the American Academy of Dermatology Volume 31, Number 2, Part 2
tion, distant dissemination may occur by lymphatic or hematogenous emboli or by lymphatic spread to regional lymph nodes.l The scalp is a relatively common site of cutaneous metastases. In one series the scalp was the site in 4% of all skin metastases." This may be because the scalp provides a vascular, immobile environment, suitable for the development of tumor cells? Brownstein and Helwig" found the source of the primary tumor was usually the lung or the kidney in men, and the metastasis appeared early in the course of the disease. In women the most common source was the breast, and the metastasis occurred late in the disease. Itis well recognized that breast carcinoma can recur long after initial treatment. Bureau et al. 7 reported a case of nodular metastases to abdominal wall skin 35 years after surgery for breast carcinoma. In our patient the scalp metastasis occurred 15 years after the diagnosis of breast adenocarcinoma. Alternatively, the scalp metastasis may be the initial sign of malignant disease," Other reported sources and types of tumor that metastasize to the scalp include the ovaries, uterus, bladder, gallbladder, prostate, testis," gastrointestinal tract, I melanoma, lymphoma, and leukemia. Distant spread of breast cancer occurs most commonly to the bone, liver, and lung. The most frequent man ifestation of scalp metastases is the occurrence of single or multiple nontender nodules'? that usually appear suddenly and grow rapidly. A well-recognized but rarer presentation is alopecia neoplastica, which is seen as single or multiple areas of scarring alopecia. Whether cictatricial alopecia occurs depends on the tumor type, duration, and host response. Invasive breast carcinoma is lobular in 10% of cases. The pattern of infiltration is diffuse and characterized by single-cell infiltration , often in single file, arranged between bundles of collagen.II The scalp metastasis in our patient demonstrated similar features. A unique feature of lobular invasivecarcinoma is that the collagen may be increased, leading to production of a scirrhous breast cancer with a firm texture on palpation. This pattern can also be seen in alopecia neoplastica. Histopathologic examination of the scalp in alopecia neoplastica demonstrates metastatic carcinoma cells in a dense collagenous stroma, with loss of pilosebaceous units.' Itis difficultto explain the complete disappearance of the pilosebaceous unit. Loss of hair follicles may be the result of fibrosis; Cohen et al. l 2 considered this to be the major mechanism.
Mallon and Dawber
321
We consider our case unusual because hair follicles appeared normal despite their presence in a dense carcinomatous infiltrate. Lobular breast carcinoma usually elicits a reactive fibrosis that leads to cicatricial alopecia. To our knowledge, the pattern without alopecia has not been described in the literature. Perhaps our case represents an early phase of an ultimately scarring alopecia that would have progressed if left untreated. Gates' stated that cutaneous metastases may not be an ominous sign because the behavior of skin tumors does not necessarily reflect the rate of growth of the primary tumor. Many women with metastatic disease in the bones have a prolonged clinical course, often more than 10 years after the identification of metastases. It Others consider that cutaneous metastases indicate a poor prognosis because they usually occur as a late phenomenon' In our patient the development of metastatic deposits in the scalp was part of progressive malignant disease, concomitant with dissemination to multiple bone sites.
REFERENCES I. Gates O. Cutaneous metastases of malignant disease. Am J Cancer 1937;30:718-30. 2. Fa y T, Henry GC. Correlation of segmental body temperature and its relation to the location of carcinomatous metastasis: clinical observations and response to methods of refrigeration. Surg Gynecol Obstet 1938;66:512-24. 3. Lever WF, Schaumburg-Lever G. Metastatic carcinoma and carcinoid. In: Histopathology of the skin. 7th ed. Philadelphia: Lippincott, 1990:651-9. 4. Abrams HL, Spiro R, Goldslein N . Metastases in carcinoma. Analysis of 1000 autopsied cases. Cancer 1950;3:7485. 5. Mehregan AH. Metastatic carcinoma to the skin. Dermatologica 1961;123:311-25. 6. Brownstein MH, Helwig EB. Patterns of cutaneous metastasis. Arch Dermatol 1972;105:862-8. 7. Bureau Y, Rodat G, Barriere H, et al. Me tastases cutanees 35 ans apres l'operation d'uncancerdu sein (3 cas). Bull Soc Fr Dermatol Syph 1965;72:412. 8. Schorr WF, Swanson PM, Gomez F, et al. Alopecia neoplastica. lAMA 1970;213:1335-7. 9. Schiff BL. Tumours of testis with cutaneous metastases to the scalp. Arch Derm ato1 Syph 1955;71:465-7. 10. Rook A, Dawber R, eds. C arcinoma met astatic to the scalp. In: Diseases of the hair and scalp. 2nd ed. Oxford: Blackwell Scientific Publications, 1991:583-4. 11. Anderson TJ, Page D. Breast structure, biology and pathology. In: McGee JO'D, Isaacson PG, Wright N, eds. Oxford textbook of pathology. Oxford: Oxford University Press, 1992:1643-8 I. 12. Cohen I, Levy E, Schreiber H. Alopecia neoplastica due to breast carcinoma. Arch Dermatol 1961;84:490-2.
3. Kishimoto TK, Springer T A. Human leukocyte adhesion deficiency:molecular basis for a defective immune response to infections of the skin. Curr Probl DermatoI1989;18:10615. 4. O'Gorman MRG, McNally AC, Anderson DC, et al. A rapid whole blood lysis technique for the diagnosis of moderate or severe leukocyte adhesion deficiency (LAD). Ann NY Acad Sci 1993;677:427-30. 5. Anderson DC, Schmalsteig FC, Finegold MJ, et at. The severe and moderate phenotypes of heritable Mac-I, LFA-I deficiency: their quantitative definition and relation to leukocyte dysfunction and clinical features. J Infect Dis 1985; 152:668-89. 6. Waldrop TC, Anderson DC, Hallmon WW, et aL Periodontal manifestations of the heritable Mac-I, LFA-I, de-
Mallon and Dawber ficiency syndrome: clinical, histopathologic and molecular characteristics. J Periodontol 1987;58:400-16. 7. Fischer A, Descamps-Latascha B, Gerita I, et aL Bone-marrow transplantation for inborn error of phagocytic cells associated with defective adherence, chemotaxis, and oxidative response during opsonized particle phagocytosis. Lancet 1983;2:473-6. 8. Le Diest F, Blanche S, Keable H, et al. Successful HLA nonidentical bone marrow transplantation in three patients with the leukocyte adhesion deficiency. Blood 1989;74:51216. 9. Weening RS, Bredius RG, WolfH, et al. Prenatal diagnostic procedure for leukocyte adhesion deficiency. Prenatal Diagn 1991;11:193-7.
Alopecia neoplastica without alopecia: A unique presentation of breast carcinoma scalp metastasis Eleanor Mallon, MBBS, MRCP, and Rodney P. R. Dawber, MA, MBChB, FRCP
Oxford, United Kingdom The scalp is a relatively common site of cutaneous metastasis. The usual presentation is of single or multiple firm scalp nodules. A well-recognized but rarer presentation is alopecia neoplastica that is seenas singleor multipleareas of cicatricial alopecia.We describea unique presentation of scalp metastasis in a patient with breast carcinoma in whom hair loss was clinically inconspicuous. The metastasis responded to combination chemotherapy. (J AM ACAD DERMATOL
1994;31:319-21.)
Cutaneous metastasis from an underlying breast carcinoma is well recognized; the scalp is a relatively common site, possibly because it provides a vascular, immobile environment, suitable for the development of tumor cells. 1, 2 Scalp metastasis may be the first sign of internal malignancy or it may occur many years after the diagnosis of malignancy. The usual presentation is of single or multiple firm scalp nodules that grow rapidly. Alopecia neop1astica is a rare form of cutaneous metastasis and can simulate other causes of alopecia. It initially appears as single or multiple areas of scarring alopecia. Whether this type of response occurs depends on tumor type, duration, and host response. Histologic study of the From the Department of Dermatology, Churchill Hospital. Reprint requests: Eleanor Mallon, MBBS, MRCP, Department of Dermatology, Churchill Hospital, Oxford, Ox3 7U, United Kingdom. Copyright @ 1994 by the American Academy of Dermatology, Inc.
0190-9622/94 $3.00 + 0
16/4/53735
scalp in alopecia neop1astica demonstrates metastatic carcinoma cells in a dense collagenous stroma, with loss of pilosebaceous units. 3 CASE REPORT
A 71-year-old woman had a left total mastectomy and axillary node dissection for adenocarcinoma in 1977. Histopathologic examination showed a lobular invasive carcinoma, and axillary nodes were free of tumor. The patient received a course of radiotherapy. Lymphedema of the left arm developed 2 years after surgery. The disease was clinicallyquiescent until 1991 when the patient reported back and hip pain. A bone scan confirmed the presence of metastasic deposits in the sternum, lumbar vertebrae, and right neck of femur. Symptomatic improvement wasobtained with localized radiotherapy, and tamoxifen therapy, 20 mg daily, was started. Nine months later an erythematous area that involved one half of the scalp had developed gradually during a 4-week period. Examination showed a 12 em X 10 em "granulomatous" plaque involving the right frontotemporal and parietal scalp (Fig. I). There was an area of
319
320
Mallon and Dawber
Journal of the American Academy of Dermatology August 1994
Fig. 1. Metastatic breast carcinoma of scalp. Erythematous plaque is present on right side of scalp; hair loss is inconspicuous.
Fig. 2. Scalp biopsy specimen shows diffuseinfiltrate of small, hyperchromatic carcinomatous cells. Normal hair follicles are present. (Hematoxylin-eosin stain, X25.) crusting in the involvedscalp. No hair loss was apparent clinically, and the hair follicles appeared normal with respect to their size, number, and appearance. A biopsy specimen from the scalp revealed that the dermis was diffusely infiltrated by ribbons and clusters of carcinomatous cells. The infiltrating cells were relatively small, hyperchromatic, and had a high nuclear /cytoplasmic ratio (Fig. 2). In addition, carcinomatous cells dissected between bundles of collagen in files. Normal hair follicles were observed, and there was no associated fibroplasia. The patient received six pulses of combination cytotoxic chemotherapy in 12 weeks; the scalp plaque resolved
after the third pulse. However, total nonscarring alopecia of the anagen effluviumtype developed as a result of the chemotherapy.
DISCUSSION Cutaneous metastasis from an underlying carcinoma is not unusual, but it is rare in comparison with metastases to other organs of the body. The overall incidence of cutaneous metastases is 2.7% 1 to 4.4%. 4 The metastasis may occur by direct extension to the skin, extension through lymphatics, or by accidental implantation during a surgical procedure. In addi-
Journal of the American Academy of Dermatology Volume 31, Number 2, Part 2
tion, distant dissemination may occur by lymphatic or hematogenous emboli or by lymphatic spread to regional lymph nodes.l The scalp is a relatively common site of cutaneous metastases. In one series the scalp was the site in 4% of all skin metastases." This may be because the scalp provides a vascular, immobile environment, suitable for the development of tumor cells? Brownstein and Helwig" found the source of the primary tumor was usually the lung or the kidney in men, and the metastasis appeared early in the course of the disease. In women the most common source was the breast, and the metastasis occurred late in the disease. Itis well recognized that breast carcinoma can recur long after initial treatment. Bureau et al. 7 reported a case of nodular metastases to abdominal wall skin 35 years after surgery for breast carcinoma. In our patient the scalp metastasis occurred 15 years after the diagnosis of breast adenocarcinoma. Alternatively, the scalp metastasis may be the initial sign of malignant disease," Other reported sources and types of tumor that metastasize to the scalp include the ovaries, uterus, bladder, gallbladder, prostate, testis," gastrointestinal tract, I melanoma, lymphoma, and leukemia. Distant spread of breast cancer occurs most commonly to the bone, liver, and lung. The most frequent man ifestation of scalp metastases is the occurrence of single or multiple nontender nodules'? that usually appear suddenly and grow rapidly. A well-recognized but rarer presentation is alopecia neoplastica, which is seen as single or multiple areas of scarring alopecia. Whether cictatricial alopecia occurs depends on the tumor type, duration, and host response. Invasive breast carcinoma is lobular in 10% of cases. The pattern of infiltration is diffuse and characterized by single-cell infiltration , often in single file, arranged between bundles of collagen.II The scalp metastasis in our patient demonstrated similar features. A unique feature of lobular invasivecarcinoma is that the collagen may be increased, leading to production of a scirrhous breast cancer with a firm texture on palpation. This pattern can also be seen in alopecia neoplastica. Histopathologic examination of the scalp in alopecia neoplastica demonstrates metastatic carcinoma cells in a dense collagenous stroma, with loss of pilosebaceous units.' Itis difficultto explain the complete disappearance of the pilosebaceous unit. Loss of hair follicles may be the result of fibrosis; Cohen et al. l 2 considered this to be the major mechanism.
Mallon and Dawber
321
We consider our case unusual because hair follicles appeared normal despite their presence in a dense carcinomatous infiltrate. Lobular breast carcinoma usually elicits a reactive fibrosis that leads to cicatricial alopecia. To our knowledge, the pattern without alopecia has not been described in the literature. Perhaps our case represents an early phase of an ultimately scarring alopecia that would have progressed if left untreated. Gates' stated that cutaneous metastases may not be an ominous sign because the behavior of skin tumors does not necessarily reflect the rate of growth of the primary tumor. Many women with metastatic disease in the bones have a prolonged clinical course, often more than 10 years after the identification of metastases. It Others consider that cutaneous metastases indicate a poor prognosis because they usually occur as a late phenomenon' In our patient the development of metastatic deposits in the scalp was part of progressive malignant disease, concomitant with dissemination to multiple bone sites.
REFERENCES I. Gates O. Cutaneous metastases of malignant disease. Am J Cancer 1937;30:718-30. 2. Fa y T, Henry GC. Correlation of segmental body temperature and its relation to the location of carcinomatous metastasis: clinical observations and response to methods of refrigeration. Surg Gynecol Obstet 1938;66:512-24. 3. Lever WF, Schaumburg-Lever G. Metastatic carcinoma and carcinoid. In: Histopathology of the skin. 7th ed. Philadelphia: Lippincott, 1990:651-9. 4. Abrams HL, Spiro R, Goldslein N . Metastases in carcinoma. Analysis of 1000 autopsied cases. Cancer 1950;3:7485. 5. Mehregan AH. Metastatic carcinoma to the skin. Dermatologica 1961;123:311-25. 6. Brownstein MH, Helwig EB. Patterns of cutaneous metastasis. Arch Dermatol 1972;105:862-8. 7. Bureau Y, Rodat G, Barriere H, et al. Me tastases cutanees 35 ans apres l'operation d'uncancerdu sein (3 cas). Bull Soc Fr Dermatol Syph 1965;72:412. 8. Schorr WF, Swanson PM, Gomez F, et al. Alopecia neoplastica. lAMA 1970;213:1335-7. 9. Schiff BL. Tumours of testis with cutaneous metastases to the scalp. Arch Derm ato1 Syph 1955;71:465-7. 10. Rook A, Dawber R, eds. C arcinoma met astatic to the scalp. In: Diseases of the hair and scalp. 2nd ed. Oxford: Blackwell Scientific Publications, 1991:583-4. 11. Anderson TJ, Page D. Breast structure, biology and pathology. In: McGee JO'D, Isaacson PG, Wright N, eds. Oxford textbook of pathology. Oxford: Oxford University Press, 1992:1643-8 I. 12. Cohen I, Levy E, Schreiber H. Alopecia neoplastica due to breast carcinoma. Arch Dermatol 1961;84:490-2.