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Merkel cell tumor of the skin
CORRESPONDENCE Merkel Cell Tumor of the Skin
is what factors contribute to a higher incidence of follicular lymphomas in Americans compared with Chinese (and residents of Nebraska), but not what are the underlying factors for aggressive lymphomas. We are also rather skeptical of the etiological role of HTLV-1 in T-cell lymphomas of Chinese as a whole though it may play a role in selected locations (such as Northeastern Taiwan, which is geographically and historically related to Southern J a p a n ) ) ' We have not e n c o u n t e r e d any e x a m p l e of a d u l t T-cell l y m p h o m a / leukemia a m o n g over 300 cases of malignant lymphoma in H o n g Kong. It is also noteworthy that in another study from China, HTLV-1 seropositivity is found in only one women, the wife of a Japanese HTLV-I carrier, among 6,684 subjects. 7 C. S. NG, MBBS, MRCPath* J. K. C. CHAN, MBBSt Institute of Pathology Caritas Medical Centre* Q u e e n Elizabeth HospitaD Hong Kong 1. Harrington DS, Ye Y, WeisenburgerDD, et al: Malignantlymphomain Nebraska and Guangzhou,China:a Comparativestudy. HuMD,THOL 18:924, 1987 2. Ng CS, ChanJKC, Lo STH, et al: Immunophenotypicanalysisof nonHodgkin'slymphomasin Chinese, a study of 75 cases in Hong Kong. Pathology 18:4t9, 1986 3. Ng CS, ChanJKC, Lo STH: Expressionof natural killercell markers in non-Hodgkin'slymphomas.HuM PATnOI.18:1257, 1987 4. Xu L, Yu L, Liu Y, et al: Phenotypicexpressionof non-Hodgkin'slymphomas in China.J Natt Cancer Inst 73:635, 1984. 5. Su IJ, Shih LY, Kadin ME, et al: Pathologic and immunologiccharacterization of malignantlymphomain Taiwan, with special reference to retrovirus-associatedadult T-celllymphoma/leukemia.Am J Clin Pathol 84:715, 1985 6. Pan I, Chung C, Komoda H, et al: Seroepidemiologyof adult T-cell leukemia virus in Taiwan.Jpn J Cancer Res (Gann)76:9, 1985 7. ZengY, Lan XY, FangJ, et al: HTLV antibodyin China. Lanceti:799, 1984
IgA Nephropathy and Membranous Nephropathy Associated With Hepatitis B Surface Antigenemia To the Editor:--Lai et al, in their recent article on mixed IgA a n d m e m b r a n o u s nephropathy associated with hepatitis B surface antigenemia, state in the abstract that theirs is the first report of such a mixed glomerulonephropathy in a patient who was a carrier of HBV. 1 This is not quite correct, since a case of mixed IgA and m e m b r a n o u s nephropathy was described previously in a 40-year-old male HBV carrier, 2 As in the case described by Lai et al, this man's glomeruli contained IgA mesangial and IgG epimembranous deposits. Hepatitis B surface Ag was detected in both sites. No attempt was made to detect either HBC Ag or HBe Ag. It would appear that this particular combination of findings in HBV carriers is rare but the recent publication of two cases with this mixed nephropathy suggests the possibility that its prevalence may be increasing.
To the Editor:--We read with great interest the recent article by Drijkoningen et al 1 concerning the diagnosis of Merkel cell carcinoma in 12 elderly patients. We agree that Merkel cell tumors share many characteristic features with other n e u r o e n d o c r i n e tumors, and o u r experience suggests that diagnosis may at times be difficult. Recently, we were involved in the case of an elderly man who experienced progressive right hemiparesis and dysphasia. General examination revealed discrete right anterior cervical and left axillary masses, a n d computed tomography of the head revealed a left parietal ring enhancing mass with associated edema. I n addition, chest and abdominal CT revealed bilateral axillary masses, left hilar adenopathy, a mass inferolateral to the right lobe of the liver, and another in the region of the left adrenal gland. Axillary node biopsy confirmed the presence of a small cell n e u r o e n d o c r i n e carcinoma. Normal lymph node architecture was replaced by sheets a n d cords of small hyperchromatic t u m o r cells, with cytologic features supporting the diagnosis of Merket cell t u m o r (Fig 1). I n fact, this was the initial working diagnosis. T h e patient improved with dexamethasone and whole brain radiation therapy, but declined c h e m o t h e r a p y a n d died with d i s s e m i n a t e d metastases four months after the onset of his illness. We were surprised to note cerebral metastasis as an initial clinical manifestation of Merkel cell carcinoma, as this had not been previously described, a n d would represent a distinctly u n u s u a l presentation of this n e u r o e n d o crine tumor. I n fact, t h r o u g h approximately 100 cases of Merkel cell carcinoma have been reported to date, these include only two cases of suspected brain metastases. 2 However, the trabecular morphology of the small cell t u m o r in this case, and the ultrastructural evidence of its n e u r o e n docrine nature, p r o m p t e d the suggestion that it was in fact a Merkel cell tumor. Yet, a careful search revealed no primary t u m o r of the skin in our patient. Instead, there was C T evidence of widespread metastases, including t u m o r in the region of the left hilum. This raised the more likely possibility that the biopsied t u m o r represented a metastasis of a small cell neuroendocrine t u m o r of b r o n c h o p u l m o n a r y origin, which in this case demonstrated trabecular morphology, absence of cellular crushing, and cytoplasmic processes. Immunohistochemical studies including staining for neuron-specific enolase were not performed. However, as D r i j k o n i n g e n et al point out, the presence of n e u r o n specific enolase is not a specific finding, though it supports the n e u r o e n d o c r i n e differentiation in Merkel cell tumors.1 I n conclusion, though the diagnosis of Merkel cell carcinoma may be suggested on the basis of light and electron microscopic features, we would caution against offering this diagnosis in the absence of a known primary t u m o r of skin.
GARY P. KAeLAN, MD, PhD MICHAELJ. BOOKBINDER, MD DOUGLAS R. HOOD, MS SAMUEL L. BRIDGERS, MD Departments of Neurology and Pathology Yale University School of Medicine New Haven, Connecticut VA Medical Center West Haven, Connecticut
ALEX MAGIL, MD Department of Pathology and Laboratory Medicine Shaughnessy Hospital Vancouver, British Columbia 1. Lai KN, Lai FM-M,Lo STH, et al: IgA nephropathyand membranous nephropathy associated with hepatitis B surface antigenemia. He-M Pa'rHOL 18:441, 1987 2. MagilA, WebberD, Chan V: Glomerulonephritisassociated with hepatitis B surface antigenemia:Report of a case with features of both membranousand IgA nephropathy. Nephron 42:335, 1986
615
1. DrijkoningenM, De Wolf-PeetersC, Van LimbergenE, et al: Merkelcell tumor of the skin: an immunohistochemicalstudy. Hu~ PATHOL 17:301, 1986 2. WickMR, GoellnerJR, ScheithauerBW, et al: Primaryneuroendocrine carcinomasof the skin (Merkelcell tumors): A clinical,histologic,and ultrastructural study of thirteen cases. Am J Clin Pathol 79:6, 1983
is what factors contribute to a higher incidence of follicular lymphomas in Americans compared with Chinese (and residents of Nebraska), but not what are the underlying factors for aggressive lymphomas. We are also rather skeptical of the etiological role of HTLV-1 in T-cell lymphomas of Chinese as a whole though it may play a role in selected locations (such as Northeastern Taiwan, which is geographically and historically related to Southern J a p a n ) ) ' We have not e n c o u n t e r e d any e x a m p l e of a d u l t T-cell l y m p h o m a / leukemia a m o n g over 300 cases of malignant lymphoma in H o n g Kong. It is also noteworthy that in another study from China, HTLV-1 seropositivity is found in only one women, the wife of a Japanese HTLV-I carrier, among 6,684 subjects. 7 C. S. NG, MBBS, MRCPath* J. K. C. CHAN, MBBSt Institute of Pathology Caritas Medical Centre* Q u e e n Elizabeth HospitaD Hong Kong 1. Harrington DS, Ye Y, WeisenburgerDD, et al: Malignantlymphomain Nebraska and Guangzhou,China:a Comparativestudy. HuMD,THOL 18:924, 1987 2. Ng CS, ChanJKC, Lo STH, et al: Immunophenotypicanalysisof nonHodgkin'slymphomasin Chinese, a study of 75 cases in Hong Kong. Pathology 18:4t9, 1986 3. Ng CS, ChanJKC, Lo STH: Expressionof natural killercell markers in non-Hodgkin'slymphomas.HuM PATnOI.18:1257, 1987 4. Xu L, Yu L, Liu Y, et al: Phenotypicexpressionof non-Hodgkin'slymphomas in China.J Natt Cancer Inst 73:635, 1984. 5. Su IJ, Shih LY, Kadin ME, et al: Pathologic and immunologiccharacterization of malignantlymphomain Taiwan, with special reference to retrovirus-associatedadult T-celllymphoma/leukemia.Am J Clin Pathol 84:715, 1985 6. Pan I, Chung C, Komoda H, et al: Seroepidemiologyof adult T-cell leukemia virus in Taiwan.Jpn J Cancer Res (Gann)76:9, 1985 7. ZengY, Lan XY, FangJ, et al: HTLV antibodyin China. Lanceti:799, 1984
IgA Nephropathy and Membranous Nephropathy Associated With Hepatitis B Surface Antigenemia To the Editor:--Lai et al, in their recent article on mixed IgA a n d m e m b r a n o u s nephropathy associated with hepatitis B surface antigenemia, state in the abstract that theirs is the first report of such a mixed glomerulonephropathy in a patient who was a carrier of HBV. 1 This is not quite correct, since a case of mixed IgA and m e m b r a n o u s nephropathy was described previously in a 40-year-old male HBV carrier, 2 As in the case described by Lai et al, this man's glomeruli contained IgA mesangial and IgG epimembranous deposits. Hepatitis B surface Ag was detected in both sites. No attempt was made to detect either HBC Ag or HBe Ag. It would appear that this particular combination of findings in HBV carriers is rare but the recent publication of two cases with this mixed nephropathy suggests the possibility that its prevalence may be increasing.
To the Editor:--We read with great interest the recent article by Drijkoningen et al 1 concerning the diagnosis of Merkel cell carcinoma in 12 elderly patients. We agree that Merkel cell tumors share many characteristic features with other n e u r o e n d o c r i n e tumors, and o u r experience suggests that diagnosis may at times be difficult. Recently, we were involved in the case of an elderly man who experienced progressive right hemiparesis and dysphasia. General examination revealed discrete right anterior cervical and left axillary masses, a n d computed tomography of the head revealed a left parietal ring enhancing mass with associated edema. I n addition, chest and abdominal CT revealed bilateral axillary masses, left hilar adenopathy, a mass inferolateral to the right lobe of the liver, and another in the region of the left adrenal gland. Axillary node biopsy confirmed the presence of a small cell n e u r o e n d o c r i n e carcinoma. Normal lymph node architecture was replaced by sheets a n d cords of small hyperchromatic t u m o r cells, with cytologic features supporting the diagnosis of Merket cell t u m o r (Fig 1). I n fact, this was the initial working diagnosis. T h e patient improved with dexamethasone and whole brain radiation therapy, but declined c h e m o t h e r a p y a n d died with d i s s e m i n a t e d metastases four months after the onset of his illness. We were surprised to note cerebral metastasis as an initial clinical manifestation of Merkel cell carcinoma, as this had not been previously described, a n d would represent a distinctly u n u s u a l presentation of this n e u r o e n d o crine tumor. I n fact, t h r o u g h approximately 100 cases of Merkel cell carcinoma have been reported to date, these include only two cases of suspected brain metastases. 2 However, the trabecular morphology of the small cell t u m o r in this case, and the ultrastructural evidence of its n e u r o e n docrine nature, p r o m p t e d the suggestion that it was in fact a Merkel cell tumor. Yet, a careful search revealed no primary t u m o r of the skin in our patient. Instead, there was C T evidence of widespread metastases, including t u m o r in the region of the left hilum. This raised the more likely possibility that the biopsied t u m o r represented a metastasis of a small cell neuroendocrine t u m o r of b r o n c h o p u l m o n a r y origin, which in this case demonstrated trabecular morphology, absence of cellular crushing, and cytoplasmic processes. Immunohistochemical studies including staining for neuron-specific enolase were not performed. However, as D r i j k o n i n g e n et al point out, the presence of n e u r o n specific enolase is not a specific finding, though it supports the n e u r o e n d o c r i n e differentiation in Merkel cell tumors.1 I n conclusion, though the diagnosis of Merkel cell carcinoma may be suggested on the basis of light and electron microscopic features, we would caution against offering this diagnosis in the absence of a known primary t u m o r of skin.
GARY P. KAeLAN, MD, PhD MICHAELJ. BOOKBINDER, MD DOUGLAS R. HOOD, MS SAMUEL L. BRIDGERS, MD Departments of Neurology and Pathology Yale University School of Medicine New Haven, Connecticut VA Medical Center West Haven, Connecticut
ALEX MAGIL, MD Department of Pathology and Laboratory Medicine Shaughnessy Hospital Vancouver, British Columbia 1. Lai KN, Lai FM-M,Lo STH, et al: IgA nephropathyand membranous nephropathy associated with hepatitis B surface antigenemia. He-M Pa'rHOL 18:441, 1987 2. MagilA, WebberD, Chan V: Glomerulonephritisassociated with hepatitis B surface antigenemia:Report of a case with features of both membranousand IgA nephropathy. Nephron 42:335, 1986
615
1. DrijkoningenM, De Wolf-PeetersC, Van LimbergenE, et al: Merkelcell tumor of the skin: an immunohistochemicalstudy. Hu~ PATHOL 17:301, 1986 2. WickMR, GoellnerJR, ScheithauerBW, et al: Primaryneuroendocrine carcinomasof the skin (Merkelcell tumors): A clinical,histologic,and ultrastructural study of thirteen cases. Am J Clin Pathol 79:6, 1983