Journal of the Royal Society of Medicine Volume 84 August 1991 myxomas are illustrated in our patient6, although the development of (cerebral) metastgses from this 'benign' tumour is rare7 8. Cardiac myxomas have led to the death, of one-quarter of patients with Carney's complex, and to serious disability in a similar proportion2. References 1 Russell B. History of Western philosophy, 2nd edn. London: George Allen & Unwin, 1946:462-3 2 Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VLW. The complex of myxomas, spotty pigmentation and endocrine overactivity. Medicine 1985;64:270-83 3 Young WF, Carney JA, Musa BU, Wulffraat NM, Lens LW, Drexhage HA. Familial Cushing's syndrome due to primary pigmented nodular adrenocortical disease. N Engi J Med 1989; 321:1659-64
Renal cell carcinoma presenting in the skin
P Cuckow FRCS P Doyle FRCS Department of Urology, Addenbrookes Hospital, Hills Road, Cambridge CB2 2QQ Keywords: skin; kidney; metastases
Infrequently renal carcinoma presents with skin metmstases which may look benign, and are missecby unwary clinicians. We present three such cases and discuss their management.
497
4 Carney JA, Hruska LS, Beauchamp GD, Gordon H. Dominant inheritance. of the complex of myxomas, spotty pigmentation and endocrine,overactivity. Mayo Clin Proc 1986;61:165-72 5 Carney JA. Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 1990; 14:206-22 6 Gorman P, Langton-Hewer R. Stroke due to atrial myxoma in a young woman with co-existing acoustic neuroma and pituitary disease (letter). J Neurol Neurosurg Psychiatry 1985;48:718-19 7 In Sook Seo, Warner TFCS, Colyer RA, Winkler RF. Metastasizing atrial myxoma. Am J Surg Pathol 1980;4:391-9 8 Bazin A, Perruzzi P, Baudrillard JC, Pluot M, Rousseaux P. Myxome cardiaque avec metastases cerebrales. Neurochirurgie 1987;33:487-9
(Accepted 26 February 1991)
Case reports Case 1 A 77-year-old woman presented to her GP with a rapidly growing chest lesion, first noticed 6 weeks previously. She attended purgical outpatients where the superficial, 2 cm, irregular, firm lesion was labelled a neurofibroma and later excised under local anaesthetic. It appeared benign and, although histology was sent, she was discharged. Four months later, after it regrew, the histology report of 'malignant clear cells most likely of renal origin' was discovered. In the next clinic no symptoms, signs or haematuria were found, although ultrasound and CT scans revealed a localized left renal tumour. She had a radical right nephrectomy and re-excision ofthe skin lesion and recovered well, histology proving identical to the original lesion (Figures 1 and 2). Lymph nodes and a bone scan were clear.
Case presented to Section of
Urology, 22 March 1990
*1~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~0
Figure 1. Low power view of the skin lesion in patient I showing renal cell carcinoma cells beneath the epidermis and dermis
Figure 2. High power view of the kidney in patient 1 showing replacement by renal cell carcinoma
0141-0768/91/ 080497-02/$02.00/0 © 1991 The Royal Society of Medicine
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Journal of the Royal Society of Medicine Volume 84 August 1991
Fifteen months later skin and breast metastases have appeared and repeat bone scan shows widespread bony involvement. She is now receiving interleukin II. Case 2 A 44-year-old man presented to his GP with a 6 mm lump on his elbow which had grown over 6 months. It was removed and, because of a fleshy, vascular appearance, sent for histology. This revealed clear cells consistent with a renal primary and prompted a CT scan and urological referral. On review he was a fit man with no stigmata of renal carcinoma, although scans showed a 5 cm mass in the lower pole of the right kidney. This was excised by a radical nephrectomy and he remains disease free 12 months later. Case 3 A 70-year-old man was sent to a dermatologist with a 7 mm right shoulder lump which had appeared over 2 weeks and was deep red and tethered. It was excised and labelled a secondary tumour until histology showed clear cells pathognomonic of renal carcinoma. No clinical stigmata were subsequently found and, after discussion with a urologist, no action was taken. Two months later he represented in Accident & Emergency with a painful, swollen arm and X-rays showed pathological fracture of the humerus and multiple pulmonary metastases. He is receiving palliation with medroxyprogesterone. Discussion Three to 11% of renal carcinomas metastasize to skin12, about 7% of all skin metastases. Up to 20% of these will be the presenting feature'2. They are distributed throughout the body, commonest in the head and neck2, frequently appear benign and 50% are misdiagnosed. They may be smooth, solitary, rapidly growing intradermal lumps which often recur2 (case 1) and ulcerate2 although subcutaneous infiltration2 and 'sebaceous horns'3 are reported. Suspicion is raised by rapid growth and atypical appearance. Histologically, cells characteristic of the underlying renal carcinoma4 are found. Weiss5 showed 75% of patients with skin involvement had lung metastases at postmortem, and Hellsten6 demonstrated 82% of metastases involve multiple sites. Twenty-five to 57% of patients with renal carcinoma have metastases at presentation and this is of prime prognostic significance7. Their treatment is uncertain and prognosis poor, with survival of 26% at one year and only 4% at 3 years8. The place of nephrectomy is unclear, although many justifications are offered. Nephrectomy controls primary related symptoms, be they local (pain, haematuria, pressure) or systemic (hormonal). These are only found in 28% of patients9 and medical management may obviate surgery. Metastases are solitary in 1.6-3.6% of patients'0 and excision of these with nephrectomy improves survival", which is between 29% and 50% at 5 years'2. Middleton"8 shows prognosis improves if the metastasis is in soft tissues and presents late. Spontaneous remission of renal carcinoma is recognized, in which case debulking nephrectomy may promote this.
It is rare (0.3-0.8% of cases) and has occurred without previous nephrectomy 3, the mortality of which is 5-6%'8. Interestingly, spontaneous regression of skin metastases is reported14. In combined treatment, both nephrectomy and available treatment modalities must be proven. Renal carcinoma is unresponsive to both chemotherapy and radiotherapy. Although medroxyprogesterone acetate palliates some cases, there are side effects and survival remains unchanged. Response to interferon is about 15% and probably depends on tumour mass, but its role is unclear'5. Interleukin II is still experimental, although early results seem promising and offer hope to future patients'6. Renal carcinoma rarely presents with skin metastases which mimic benign lesions and are easily misdiagnosed. Histology must always be sent on excised skin lesions. The prognosis for patients who present with skin and other metastases is poor and nephrectomy rarely indicated. Until this serendipitous tumour can be better understood and treated, the management of metastatic renal carcinoma will depend on the preferences of the individual patient and surgeon. References 1 Rosenthal AL, Lever WF. Involvement of the skin in renal carcinoma. Arch Dermatol 1957;76:96 2 Lumpkin LR, Tschen JA. Renal cell carcinoma metastatic to skin. Cutis 1984;34:143 3 Peterson JL, McMarlin SL. Metastatic renal cell carcinoma presenting as a cutaneous horn. J Dermatol Surg Oncol 1983;9:815 4 Brownstein MH, Helwig EB. Metastatic tumours of the skin. Cancer 1972;29:1298 5 Weiss L, Harlos JP, Torhorst J, et aL Metastatic patterns of renal carcinoma: An analysis of 687 necropsies. Cancer Res Clin Oncol 1988;114:605 6 Hellsten S, Berge T, Linel F. Clinically unrecognised renal carcinoma: aspects of tumour morphology, lymphatic and haematogenous metastatic spread. Br J Urol 1983;55:166 7 Bottiger LE. Prognosis in renal carcinoma. Cancer 1970;26:780 8 Patel NP, Lavengood RW. Renal cell carcinoma: natural history and results of treatment. J Urol 1978;119:722-6 9 Montie JE, Stewart BH, Straffon RA. The role of adjunctive nephrectomy in patients with metastatic renal carcinoma. J Urol 1977;117:272 10 Skinner DG, Colvin RB, Vermillion CD, Pfister RC, Leadbetter WF. Diagnosis and management of renal cell carcinoma. A clinical and pathologic study of 309 cases. Cancer 197128:1165 11 Middleton AW. Indications for and results of nephrectomy for metastatic renal cell carcinoma. Urol Clin NAm 1980;7:711-17 12 Golimbu M, Al-Askari S, Tessler A, Morales P. Aggressive treatment of metastatic renal cancer. J Urol 1986;136:805-7 13 Freed SZ, Halperin JP, Gordon M. Idiopathic regression of metastases from renal cell carcinoma. J Urol 1977;118:538-42 14 Thomas PJ, Stott M, Royle GT. Spontaneous regression of subcutaneous and pulmonary metastases from renal carcinoma. Br J Urol 1989;63:102-3 15 Porszolt F, Messerer D, Hautmann R, et al Treatment of advanced renal cancer with recombinant interferon alpha as a single agent and in combination with medroxy progesterone acetate. J Cancer Res Clin Oncol 1988;114:95-100 16 Editorial. Interleukin II: sunrise for immunotherapy? Lancet 1989;i:308
(Accepted 7 March 1991)
Renal cell carcinoma presenting in the skin
P Cuckow FRCS P Doyle FRCS Department of Urology, Addenbrookes Hospital, Hills Road, Cambridge CB2 2QQ Keywords: skin; kidney; metastases
Infrequently renal carcinoma presents with skin metmstases which may look benign, and are missecby unwary clinicians. We present three such cases and discuss their management.
497
4 Carney JA, Hruska LS, Beauchamp GD, Gordon H. Dominant inheritance. of the complex of myxomas, spotty pigmentation and endocrine,overactivity. Mayo Clin Proc 1986;61:165-72 5 Carney JA. Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 1990; 14:206-22 6 Gorman P, Langton-Hewer R. Stroke due to atrial myxoma in a young woman with co-existing acoustic neuroma and pituitary disease (letter). J Neurol Neurosurg Psychiatry 1985;48:718-19 7 In Sook Seo, Warner TFCS, Colyer RA, Winkler RF. Metastasizing atrial myxoma. Am J Surg Pathol 1980;4:391-9 8 Bazin A, Perruzzi P, Baudrillard JC, Pluot M, Rousseaux P. Myxome cardiaque avec metastases cerebrales. Neurochirurgie 1987;33:487-9
(Accepted 26 February 1991)
Case reports Case 1 A 77-year-old woman presented to her GP with a rapidly growing chest lesion, first noticed 6 weeks previously. She attended purgical outpatients where the superficial, 2 cm, irregular, firm lesion was labelled a neurofibroma and later excised under local anaesthetic. It appeared benign and, although histology was sent, she was discharged. Four months later, after it regrew, the histology report of 'malignant clear cells most likely of renal origin' was discovered. In the next clinic no symptoms, signs or haematuria were found, although ultrasound and CT scans revealed a localized left renal tumour. She had a radical right nephrectomy and re-excision ofthe skin lesion and recovered well, histology proving identical to the original lesion (Figures 1 and 2). Lymph nodes and a bone scan were clear.
Case presented to Section of
Urology, 22 March 1990
*1~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~0
Figure 1. Low power view of the skin lesion in patient I showing renal cell carcinoma cells beneath the epidermis and dermis
Figure 2. High power view of the kidney in patient 1 showing replacement by renal cell carcinoma
0141-0768/91/ 080497-02/$02.00/0 © 1991 The Royal Society of Medicine
498
Journal of the Royal Society of Medicine Volume 84 August 1991
Fifteen months later skin and breast metastases have appeared and repeat bone scan shows widespread bony involvement. She is now receiving interleukin II. Case 2 A 44-year-old man presented to his GP with a 6 mm lump on his elbow which had grown over 6 months. It was removed and, because of a fleshy, vascular appearance, sent for histology. This revealed clear cells consistent with a renal primary and prompted a CT scan and urological referral. On review he was a fit man with no stigmata of renal carcinoma, although scans showed a 5 cm mass in the lower pole of the right kidney. This was excised by a radical nephrectomy and he remains disease free 12 months later. Case 3 A 70-year-old man was sent to a dermatologist with a 7 mm right shoulder lump which had appeared over 2 weeks and was deep red and tethered. It was excised and labelled a secondary tumour until histology showed clear cells pathognomonic of renal carcinoma. No clinical stigmata were subsequently found and, after discussion with a urologist, no action was taken. Two months later he represented in Accident & Emergency with a painful, swollen arm and X-rays showed pathological fracture of the humerus and multiple pulmonary metastases. He is receiving palliation with medroxyprogesterone. Discussion Three to 11% of renal carcinomas metastasize to skin12, about 7% of all skin metastases. Up to 20% of these will be the presenting feature'2. They are distributed throughout the body, commonest in the head and neck2, frequently appear benign and 50% are misdiagnosed. They may be smooth, solitary, rapidly growing intradermal lumps which often recur2 (case 1) and ulcerate2 although subcutaneous infiltration2 and 'sebaceous horns'3 are reported. Suspicion is raised by rapid growth and atypical appearance. Histologically, cells characteristic of the underlying renal carcinoma4 are found. Weiss5 showed 75% of patients with skin involvement had lung metastases at postmortem, and Hellsten6 demonstrated 82% of metastases involve multiple sites. Twenty-five to 57% of patients with renal carcinoma have metastases at presentation and this is of prime prognostic significance7. Their treatment is uncertain and prognosis poor, with survival of 26% at one year and only 4% at 3 years8. The place of nephrectomy is unclear, although many justifications are offered. Nephrectomy controls primary related symptoms, be they local (pain, haematuria, pressure) or systemic (hormonal). These are only found in 28% of patients9 and medical management may obviate surgery. Metastases are solitary in 1.6-3.6% of patients'0 and excision of these with nephrectomy improves survival", which is between 29% and 50% at 5 years'2. Middleton"8 shows prognosis improves if the metastasis is in soft tissues and presents late. Spontaneous remission of renal carcinoma is recognized, in which case debulking nephrectomy may promote this.
It is rare (0.3-0.8% of cases) and has occurred without previous nephrectomy 3, the mortality of which is 5-6%'8. Interestingly, spontaneous regression of skin metastases is reported14. In combined treatment, both nephrectomy and available treatment modalities must be proven. Renal carcinoma is unresponsive to both chemotherapy and radiotherapy. Although medroxyprogesterone acetate palliates some cases, there are side effects and survival remains unchanged. Response to interferon is about 15% and probably depends on tumour mass, but its role is unclear'5. Interleukin II is still experimental, although early results seem promising and offer hope to future patients'6. Renal carcinoma rarely presents with skin metastases which mimic benign lesions and are easily misdiagnosed. Histology must always be sent on excised skin lesions. The prognosis for patients who present with skin and other metastases is poor and nephrectomy rarely indicated. Until this serendipitous tumour can be better understood and treated, the management of metastatic renal carcinoma will depend on the preferences of the individual patient and surgeon. References 1 Rosenthal AL, Lever WF. Involvement of the skin in renal carcinoma. Arch Dermatol 1957;76:96 2 Lumpkin LR, Tschen JA. Renal cell carcinoma metastatic to skin. Cutis 1984;34:143 3 Peterson JL, McMarlin SL. Metastatic renal cell carcinoma presenting as a cutaneous horn. J Dermatol Surg Oncol 1983;9:815 4 Brownstein MH, Helwig EB. Metastatic tumours of the skin. Cancer 1972;29:1298 5 Weiss L, Harlos JP, Torhorst J, et aL Metastatic patterns of renal carcinoma: An analysis of 687 necropsies. Cancer Res Clin Oncol 1988;114:605 6 Hellsten S, Berge T, Linel F. Clinically unrecognised renal carcinoma: aspects of tumour morphology, lymphatic and haematogenous metastatic spread. Br J Urol 1983;55:166 7 Bottiger LE. Prognosis in renal carcinoma. Cancer 1970;26:780 8 Patel NP, Lavengood RW. Renal cell carcinoma: natural history and results of treatment. J Urol 1978;119:722-6 9 Montie JE, Stewart BH, Straffon RA. The role of adjunctive nephrectomy in patients with metastatic renal carcinoma. J Urol 1977;117:272 10 Skinner DG, Colvin RB, Vermillion CD, Pfister RC, Leadbetter WF. Diagnosis and management of renal cell carcinoma. A clinical and pathologic study of 309 cases. Cancer 197128:1165 11 Middleton AW. Indications for and results of nephrectomy for metastatic renal cell carcinoma. Urol Clin NAm 1980;7:711-17 12 Golimbu M, Al-Askari S, Tessler A, Morales P. Aggressive treatment of metastatic renal cancer. J Urol 1986;136:805-7 13 Freed SZ, Halperin JP, Gordon M. Idiopathic regression of metastases from renal cell carcinoma. J Urol 1977;118:538-42 14 Thomas PJ, Stott M, Royle GT. Spontaneous regression of subcutaneous and pulmonary metastases from renal carcinoma. Br J Urol 1989;63:102-3 15 Porszolt F, Messerer D, Hautmann R, et al Treatment of advanced renal cancer with recombinant interferon alpha as a single agent and in combination with medroxy progesterone acetate. J Cancer Res Clin Oncol 1988;114:95-100 16 Editorial. Interleukin II: sunrise for immunotherapy? Lancet 1989;i:308
(Accepted 7 March 1991)
