- Email: [email protected]
Firm, Purplish-Red Nodular Mass in the Hard Palate
J#
D ia g n o s in g O ra l D is e a s e
Firm, purplish-red nodular mass in the hard palate W illia m B. Wescott, D M D , M S R a lp h W . C orreli, DDS
A
jL JL 55-year-old m an w ith a 50pack-year history of smoking and heavy use of alcohol had shortness of breath, fatigue, and weakness. At his first hospital admission, one year pre viously, amyloidosis with congestive heart failure, sick sinus syndrome, and peripheral neuropathy were diag nosed and documented by rectal, lymph node, and pulmonary biopsy examinations. The patient received several units of packed red cells to cor rect a low hematocrit level. A review of bone marrow aspirate slides suggested that the patient could be suffering from pure red cell hypoplasia. The patient was discharged two days after admis sion with a prescription of Prednisone, 40 mg orally every morning, in addi tion to other medications. Frequent transfusions of packed red cells al lowed the patient to be followed on an outpatient basis. At the follow-up ex amination, a hyperpigmented, indu rated plaque and a nodular lesion were noted on the patient’s left leg (Fig 1). A biopsy specimen of the leg lesion was suggestive of Kaposi’s sarcoma; how ever, pyogenic granuloma could not be ruled out. A repeat biopsy examina tion was performed. Several bluish, nodular masses of unknown duration with surrounding swelling were also present in the palate (Fig 2, 3). Biop sies of these oral lesions were also done. Oral and skin lesions of another patient are shown in Figures 4-6. 1040 ■ JADA, Vol. 105, December 1982
Diagnosis Microscopic examination of the bi opsy tissue showed abundant small blood vessels lined by endothelial cells with enlarged nuclei and sup porting tissue with many spindleshaped, fibroblast-like cells, along with considerable extravasation of red cells and the presence of hemosiderin (Fig 7, 8). The diagnosis reported was Kaposi’s sarcoma. A repeat bone mar row biopsy specimen at this time showed hemosiderosis with erythroid hypoplasia and moderate lym pho cytosis. The lesions on the extremities and those in the palate received 1,000 rads in five fractions.
Characteristics Kaposi (1837-1902) first described and reported id iop ath ic m u ltip le p ig mented sarcoma of the skin in 1872.1 A lthough many other descriptive names have been used, the disease is commonly referred to as Kaposi’s sar coma. Historically, this rare malig nancy has an incidence of approxi mately 0.05 1100,000 of the popula tion in North America and Europe and constitutes 10% of all cancers seen in Central Africa.2 Kaposi’s sarcoma oc curs most commonly in 50- to 70year-old men often of Eastern, Euro pean, or Mediterranean background in this country and in Europe. The prin
cipal difference between cases in Af rica and those in other countries is the earlier age of onset. There is a greater frequency of occurrence in African c h ild re n , often w ith s ig n ific a n t adenopathy and, at times, absence of cutaneous lesions. In Africa, there is a specific geographic distribution with a high incidence in equatorial regions and a lower incidence in the tropics. Apparently no race is immune as cases have been reported from almost every country. The disease commonly appears as redd ish - b ro w n or p u r p lis h red, slightly elevated, nonulcerated, and nontender lesions in the skin of ex tremities, particularly the hands and feet. The lesions may coalesce to form large plaques or tumors that may be come eroded, ulcerated, or fungating. Oral lesions usually follow skin le sions; however, the initial presenta tion may be intraoral with the most common site the palate, followed by the tongue and lips. When the oral cav ity is involved, multiple lesions may be present. The disease may begin in lymph nodes or in any internal organ. It might be regarded as a systemic dis ease of multicentric origin, and is likely to involve, independently and in an unpredictable manner, skin and internal organs simultaneously or suc cessively.3 The disease may spread along veins and lymphatics to involve other areas. When this occurs, edema
D I A G N O S IN G
ORAL
D IS E A S E
Fig 1, left ■ Firm, palpable, purplish-red nodule and plaque on left leg.
Fig 2, right ■ Nodu lar lesions of Kaposi’s sarcoma appearing in hard palate.
Fig 3, left ■ Nodular, palatable Kaposi’s sarcoma lesions show ing biopsy examina tion sites.
Fig 4, right ■ Small, purple macule in buccal mucosa of another patient.
Fig 5, left ■ Macular, diffuse presentation in skin of hand in same patient as Figure 4.
Fig 6, right ■ Multiple, small, nodular skin lesions in leg of same patient shown in Figure 4.
Fig 7, left ■ Dilated vascular spaces, extravasated erythrocytes, and supporting stroma seen in Kaposi’s sarcoma lesion (orig mag x 200).
Fig 8, right ■ Vascular spaces among bundles of spindle-shaped fibroblasts. Note mitotic figure centrally located in section (orig mag x 400). Wescott-Correll: NODULAR MASS IN HARD PALATE ■ 1041
D IA G N O SIN G
ORAL DISEASE
is a common feature and may be re lated to plugging of the lymphatics with tumors. In other cases, Kaposi’s sarcoma may never extend beyond the primary site.2 In some cases, spon taneous involution has occurred after several years.3 Radiation therapy is w idely regarded as the preferred treatment. Prognosis for Kaposi’s sar coma varies with several factors. The average survival rate is eight to 13 years with longer rates in patients who have only cutaneous involvement. In early lesions, the microscopic changes are those of nonspecific ' granulomatous inflam m ation w ith many small, engorged, and proliferat ing capillaries and infiltrates of lym phocytes, monocytes, and other cells. The new vessels are often open-ended and imperfect so that erythrocytes es cape, le a d in g to d e p o s itio n of hemosiderin. The early nodular infil trate materially contributes to the dis tinctive histologic picture. The cells of the infiltrate are embedded in support ing tissue that appears to merge and blend with the capillary endothelial cells. The fibroblastic cells show cellu lar atypism with variation in nuclear size and in staining so that they resem ble fibrosarcoma. Whorls of spindle cells with small blood spaces between them are seen in later stages. The cytoplasm and cytoplasmic processes are not well defined, and in some areas there are solid nests of oval and spin dle cells. An interesting and puzzling feature of Kaposi’s sarcoma is the frequent as sociation w ith m alignant lymphoreticular neoplasm s, particularly Hodgkin’s disease. It is postulated that both Kaposi’s sarcoma and malignant lymphoreticular tumors are associated with a deficient immune response.4'9 Reidy and others6 reported an addi tional case of a patient w ith both Kaposi’s sarcoma, and autoimmune hemolytic anemia and discuss the pos sible relationship of Kaposi’s sarcoma and immune hemolysis. Harwood and others7reported in their study of 44 pa tients with Kaposi’s sarcoma that a major increase in the incidence of the disease was found in renal transplant recipients who were of Mediterranean or Jewish origin. Also their findings suggested a relationship between the degree of impairment of cellular im munity and the extent of the disease. There appears to be a direct relation ship between Kaposi’s sarcoma and 1042 ■ JADA» Vol. 105, December 1982
the immunodeficient state either on an iatrogenic basis or secondary to preexisting neoplasia or immune defi ciency, including autoimmune dis eases.5Also, the cluster distribution of the malignancy in Africa suggests that , heredity, infections, and environmen tal factors may have a role in the etiol ogy of the disease.10 Herpes type virus particles have been demonstrated by Giraldo and others11 in tissue culture cell lines from African cases. They also show an apparent serologic association be tween cytomegalovirus (CMV) and Kaposi’s sarcoma in European12 and Am erican13 patients. Boldogh and others14 found CMV DNA and CMV RNA in biopsy specimens of tumors in patients with Kaposi’s sarcoma. There was no association between Kaposi’s sarcoma and herpes simplex virus Type II or with Epstein-Barr virus. In contrast to the usual occurrence of Kaposi’s sarcoma in older age groups, there has been a recent outbreak (since late 1980) among young, m ain ly homosexual men in New York and Cal ifornia as well as many other states and several foreign countries. Cases have been reported in men of all racial backgrounds from 24 to 40 years of age. The disease is more aggressive than that usually seen and more closely resembles the form of Kaposi’s sarcoma seen in young Africans. By the middle of 1982 there were a total of 438 cases, w ith 330 cases among young homosexual and bisexual men, 58 in heterosexual men, 27 in men with unknown sexual orientation, and 23 cases in heterosexual women.
History of drug use A history of drug use and abuse was a common finding. By late September 1982, 593 cases of Kaposi’s sarcoma were reported, w ith 243 related deaths. These notable features have emerged: the patients are typically young, homosexual men, most of whom live in large cities and many of whom use drugs; the infectious agents are low-grade pathogens that cause opportunistic infections in com promised hosts; and the death rate is excessive.15 Pneumocystis carinii pneumonia and other serious opportunistic infec tions, including viral, microbial, fun gal, and protozoan disease have also occurred in this same group of pa
tients. The wide variety of pathogens involved in these opportunistic infec tions and the severity of the illness have suggested a compromised state w ith little or no normal defenses, m uch like that found in immunosuppressed patients. This m u lti faceted disease complex of oppor tunistic infection and Kaposi’s sar coma has been termed an Acquired Immunodeficiency Syndrome (AIDS). The factors re sp o n sib le for the immunosuppression and for devel opment of Kaposi’s sarcoma are not known. Many potential agents have been suggested. Cytomegalovirus in fection has been a common finding. CMV is shed in semen and saliva and has been shown to cause im m uno suppression in mice and human be ings.16,17 CMV has been cultured from tumor tissue, and Kaposi’s sarcoma has been linked with CMV,1214 so the virus is a candidate as the etiologic fac tor or at least one of the factors of Kaposi’s sarcoma. Cytomegalic inclu sions are most prominent in vascular e n dothelial cells in a d u lts 17 and Kaposi’s sarcoma may be derived from endothelial cells. The use of drugs has also been suggested as a possible contributing factor in the development of Kaposi’s sarcoma. Volatile nitrites may have some role in the development of the disease. Both amyl nitrite and isobutyl nitrite are commonly used (more than 86%) as sexual stimulants or recre ational drugs by homosexual men.18 Many of the heterosexual patients with Kaposi’s sarcoma also have a history of drug use. Other factors associated with h o m o s e x u a l p ra c tic e s in c lu d e mucosal exposure to seminal plasma and the use of steroid creams, both of w h ic h have im m unosuppressive properties.19 21 The patients have had profound defects in the cell-mediated immune response. All were anergic, had T-lymphocyte depletion, and had markedly depressed lymphoproliferative responses. It is im portant that dentists are aware of this increase in Kaposi’s sar coma because the disease may first occur as an oral lesion. Also, oral can didiasis, lymphadenopathy, fever, weight loss, and diarrhea may be pres ent for several months as prodromal signs and symptoms in patients with AIDS. Because Kaposi’s sarcoma is generally considered to be a disease that appears in the skin, it may not be
D IA G N O SIN G
considered in the early differential diagnosis when the first lesion appears in the oral cavity. This early oral lesion may resemble a pyogenic granuloma and may be diagnosed as such.22 Oral lesions with a vascular component should be considered as potentially dangerous until the history and histo pathologic findings prove otherwise.
Summary A brief report on Kaposi’s sarcoma as encountered in Africa and in a small segment of older patients in this and other countries is presented. The re cent outbreak over the past two years of Kaposi’s sarcoma in patients with an acquired im m unodeficiency syn drome is reviewed. Because of the in creased incidence of this disease, den tists must be familiar with the oral manifestations.
The publication of this series is coordinated by - the Veterans Administration Dental Education Center I West Coast and supported in part by funding provided by the Veterans Administration Exchange of Medical Information Program. The opinions and assertions contained herein are those of the authors and are not to be construed as official or necessarily representing the views of the Veterans Administration.
Dr. Wescott is director, Veterans Administra tion Dental Education Center, and professor, de partm ent of oral diagnosis, radiology, and pathology, Loma Linda University School of Den tistry. Dr. Correll is associate director, Veterans Administration Dental Education Center,. Los Angeles, and clinical assistant professor, depart ment of pathology, University of Southern Cali fornia School of Dentistry, Los Angeles. Address request for reprints to Dental Education Center, (161), Veterans Administration Medical Center, West Los Angeles, 90073. 1. Kaposi, M. Idiopathic multiple pigmented sarcoma of the skin. Arch Dermatol 4:265-273, 1872. 2. Mansell, P.W. Kaposi’s sarcoma— an emerg ing epidemic. Cancer Bull 34:72-74,1982. 3. Montgomery, H. Dermatopathology. New York, Harper & Row, 1967, p 1111. 4. Hajdu, S.I. Pathology of soft tissue tumors. Philadelphia, Lea & Febiger, 1979, p 401. 5. Ulbright, T.M., and Santa Cruz, D.J. Kaposi's sarcoma: relationship with hematologic, lym phoid, and thymic neoplasia. Cancer 47:963-973, 1981. 6. Reidy, T.J., and others. Kaposi’s sarcoma and immune hemolysis. Cancer 50:1297-1298, 1982. 7. Harwood, A.R., and others. Kaposi’s sar coma in recipients of renal transplants. Amer J Med 67:759-765, 1979. 8. Farman, A.G., and Uys, P.B. Oral Kaposi’s sarcoma. Oral Surg 39:288-296, 1975. 9. Haim, S., and others. Kaposi’s sarcoma in as sociation with immunosuppressive therapy. Isr J Med Sci 8:1993-1997, 1972. 10. Safai, B., and Good, R.A. Kaposi’s sarcoma: a review and recent developments. CA 3:2-12, 1981. 11. Giraldo, G.; Beth, E.; and Haguenau, F. Herpes-type virus particles in tissue culture of Kaposi’s sarcoma from different geographic re gions. J Natl Cancer Inst 49:1509-1526, 1972. 12. Giraldo, G.; Beth, E.; and Henle, W. Anti
ORAL DISEA SE
body patterns to herpes viruses in Kaposi’s sar coma: serological association of European Kaposi’s sarcoma with cytomegalovirus. Int J Cancer 15:839-848,1975. 13. Giraldo, G.; Beth, E.; and Henle, W. Anti body patterns to herpes viruses in Kaposi’s sar coma II. Serological association of American Kaposi’s sarcoma with cytomegalovirus. Int J Cancer 22:126-131, 1978. 14. Boldogh, I., and others. Kaposi’s sarcoma IV Detection of CMV DNA and CMV RNA and CMNA in tumor biopsies. IntJ Cancer 28:469-474, 1981. 15. Durack, D.T. Opportunistic infections and Kaposi’s sarcoma in homosexual men. N Eng J Med 305:1465-1467, 1981. 16. Hamilton, J.R.; Overall, J.C., Jr.; and Glas gow, L.A. Synergistic effect on mortality in mice with murine cytomegalovirus and Pseudomonas aeruginosa, StaphyJococcus aureus, or Candida albicans infections. Infect Im m un 14:982-989, 1976. 17. Carney, W.P., and others. Analysis of T ly m p h o c y te subsets in c y to m e g a lo v iru s mononucleosis. J Immunol 126:2114-2116,1981. 18. Wong, T.W., and Warner, N.E. Cytomegalic inclusion disease in adults. Arch Pathol 74:403422,1962. 19. Centers for Disease Control Task Force Re port: epidemiologic aspects of the current out break of Kaposi’s sarcoma and opportunistic in fections. N Eng J Med 306:248-252,1982. 20. Lord, E.M.; Sensabaugh, G.F.; and Stites, D.P. Immunosuppressive activity of hum an sem inal plasma. J Immunol 118:1704-1711,1977. 21. Anderson, D.J., and Tarter, T.H. Immuno suppressive effects of mouse seminal plasma components in vivo and in vitro. J Im m unol 128:535-539, 1982. 22. Neumann, H.H. Use of steroid creams as a p o s s ib le cause o f im m u n o s u p r e s s io n in homosexuals (letter). N Eng J Med 306:935,1982. 23. Breauer, M.K.; Gates, P.E.; and Doyle, J.L. Visceral Kaposi’s sarcoma presenting w ith gingi val lesions. Oral Surg 50:151-155,1980.
W escott-Correll: NODULAR M ASS IN H ARD PALATE ■ 1043
D ia g n o s in g O ra l D is e a s e
Firm, purplish-red nodular mass in the hard palate W illia m B. Wescott, D M D , M S R a lp h W . C orreli, DDS
A
jL JL 55-year-old m an w ith a 50pack-year history of smoking and heavy use of alcohol had shortness of breath, fatigue, and weakness. At his first hospital admission, one year pre viously, amyloidosis with congestive heart failure, sick sinus syndrome, and peripheral neuropathy were diag nosed and documented by rectal, lymph node, and pulmonary biopsy examinations. The patient received several units of packed red cells to cor rect a low hematocrit level. A review of bone marrow aspirate slides suggested that the patient could be suffering from pure red cell hypoplasia. The patient was discharged two days after admis sion with a prescription of Prednisone, 40 mg orally every morning, in addi tion to other medications. Frequent transfusions of packed red cells al lowed the patient to be followed on an outpatient basis. At the follow-up ex amination, a hyperpigmented, indu rated plaque and a nodular lesion were noted on the patient’s left leg (Fig 1). A biopsy specimen of the leg lesion was suggestive of Kaposi’s sarcoma; how ever, pyogenic granuloma could not be ruled out. A repeat biopsy examina tion was performed. Several bluish, nodular masses of unknown duration with surrounding swelling were also present in the palate (Fig 2, 3). Biop sies of these oral lesions were also done. Oral and skin lesions of another patient are shown in Figures 4-6. 1040 ■ JADA, Vol. 105, December 1982
Diagnosis Microscopic examination of the bi opsy tissue showed abundant small blood vessels lined by endothelial cells with enlarged nuclei and sup porting tissue with many spindleshaped, fibroblast-like cells, along with considerable extravasation of red cells and the presence of hemosiderin (Fig 7, 8). The diagnosis reported was Kaposi’s sarcoma. A repeat bone mar row biopsy specimen at this time showed hemosiderosis with erythroid hypoplasia and moderate lym pho cytosis. The lesions on the extremities and those in the palate received 1,000 rads in five fractions.
Characteristics Kaposi (1837-1902) first described and reported id iop ath ic m u ltip le p ig mented sarcoma of the skin in 1872.1 A lthough many other descriptive names have been used, the disease is commonly referred to as Kaposi’s sar coma. Historically, this rare malig nancy has an incidence of approxi mately 0.05 1100,000 of the popula tion in North America and Europe and constitutes 10% of all cancers seen in Central Africa.2 Kaposi’s sarcoma oc curs most commonly in 50- to 70year-old men often of Eastern, Euro pean, or Mediterranean background in this country and in Europe. The prin
cipal difference between cases in Af rica and those in other countries is the earlier age of onset. There is a greater frequency of occurrence in African c h ild re n , often w ith s ig n ific a n t adenopathy and, at times, absence of cutaneous lesions. In Africa, there is a specific geographic distribution with a high incidence in equatorial regions and a lower incidence in the tropics. Apparently no race is immune as cases have been reported from almost every country. The disease commonly appears as redd ish - b ro w n or p u r p lis h red, slightly elevated, nonulcerated, and nontender lesions in the skin of ex tremities, particularly the hands and feet. The lesions may coalesce to form large plaques or tumors that may be come eroded, ulcerated, or fungating. Oral lesions usually follow skin le sions; however, the initial presenta tion may be intraoral with the most common site the palate, followed by the tongue and lips. When the oral cav ity is involved, multiple lesions may be present. The disease may begin in lymph nodes or in any internal organ. It might be regarded as a systemic dis ease of multicentric origin, and is likely to involve, independently and in an unpredictable manner, skin and internal organs simultaneously or suc cessively.3 The disease may spread along veins and lymphatics to involve other areas. When this occurs, edema
D I A G N O S IN G
ORAL
D IS E A S E
Fig 1, left ■ Firm, palpable, purplish-red nodule and plaque on left leg.
Fig 2, right ■ Nodu lar lesions of Kaposi’s sarcoma appearing in hard palate.
Fig 3, left ■ Nodular, palatable Kaposi’s sarcoma lesions show ing biopsy examina tion sites.
Fig 4, right ■ Small, purple macule in buccal mucosa of another patient.
Fig 5, left ■ Macular, diffuse presentation in skin of hand in same patient as Figure 4.
Fig 6, right ■ Multiple, small, nodular skin lesions in leg of same patient shown in Figure 4.
Fig 7, left ■ Dilated vascular spaces, extravasated erythrocytes, and supporting stroma seen in Kaposi’s sarcoma lesion (orig mag x 200).
Fig 8, right ■ Vascular spaces among bundles of spindle-shaped fibroblasts. Note mitotic figure centrally located in section (orig mag x 400). Wescott-Correll: NODULAR MASS IN HARD PALATE ■ 1041
D IA G N O SIN G
ORAL DISEASE
is a common feature and may be re lated to plugging of the lymphatics with tumors. In other cases, Kaposi’s sarcoma may never extend beyond the primary site.2 In some cases, spon taneous involution has occurred after several years.3 Radiation therapy is w idely regarded as the preferred treatment. Prognosis for Kaposi’s sar coma varies with several factors. The average survival rate is eight to 13 years with longer rates in patients who have only cutaneous involvement. In early lesions, the microscopic changes are those of nonspecific ' granulomatous inflam m ation w ith many small, engorged, and proliferat ing capillaries and infiltrates of lym phocytes, monocytes, and other cells. The new vessels are often open-ended and imperfect so that erythrocytes es cape, le a d in g to d e p o s itio n of hemosiderin. The early nodular infil trate materially contributes to the dis tinctive histologic picture. The cells of the infiltrate are embedded in support ing tissue that appears to merge and blend with the capillary endothelial cells. The fibroblastic cells show cellu lar atypism with variation in nuclear size and in staining so that they resem ble fibrosarcoma. Whorls of spindle cells with small blood spaces between them are seen in later stages. The cytoplasm and cytoplasmic processes are not well defined, and in some areas there are solid nests of oval and spin dle cells. An interesting and puzzling feature of Kaposi’s sarcoma is the frequent as sociation w ith m alignant lymphoreticular neoplasm s, particularly Hodgkin’s disease. It is postulated that both Kaposi’s sarcoma and malignant lymphoreticular tumors are associated with a deficient immune response.4'9 Reidy and others6 reported an addi tional case of a patient w ith both Kaposi’s sarcoma, and autoimmune hemolytic anemia and discuss the pos sible relationship of Kaposi’s sarcoma and immune hemolysis. Harwood and others7reported in their study of 44 pa tients with Kaposi’s sarcoma that a major increase in the incidence of the disease was found in renal transplant recipients who were of Mediterranean or Jewish origin. Also their findings suggested a relationship between the degree of impairment of cellular im munity and the extent of the disease. There appears to be a direct relation ship between Kaposi’s sarcoma and 1042 ■ JADA» Vol. 105, December 1982
the immunodeficient state either on an iatrogenic basis or secondary to preexisting neoplasia or immune defi ciency, including autoimmune dis eases.5Also, the cluster distribution of the malignancy in Africa suggests that , heredity, infections, and environmen tal factors may have a role in the etiol ogy of the disease.10 Herpes type virus particles have been demonstrated by Giraldo and others11 in tissue culture cell lines from African cases. They also show an apparent serologic association be tween cytomegalovirus (CMV) and Kaposi’s sarcoma in European12 and Am erican13 patients. Boldogh and others14 found CMV DNA and CMV RNA in biopsy specimens of tumors in patients with Kaposi’s sarcoma. There was no association between Kaposi’s sarcoma and herpes simplex virus Type II or with Epstein-Barr virus. In contrast to the usual occurrence of Kaposi’s sarcoma in older age groups, there has been a recent outbreak (since late 1980) among young, m ain ly homosexual men in New York and Cal ifornia as well as many other states and several foreign countries. Cases have been reported in men of all racial backgrounds from 24 to 40 years of age. The disease is more aggressive than that usually seen and more closely resembles the form of Kaposi’s sarcoma seen in young Africans. By the middle of 1982 there were a total of 438 cases, w ith 330 cases among young homosexual and bisexual men, 58 in heterosexual men, 27 in men with unknown sexual orientation, and 23 cases in heterosexual women.
History of drug use A history of drug use and abuse was a common finding. By late September 1982, 593 cases of Kaposi’s sarcoma were reported, w ith 243 related deaths. These notable features have emerged: the patients are typically young, homosexual men, most of whom live in large cities and many of whom use drugs; the infectious agents are low-grade pathogens that cause opportunistic infections in com promised hosts; and the death rate is excessive.15 Pneumocystis carinii pneumonia and other serious opportunistic infec tions, including viral, microbial, fun gal, and protozoan disease have also occurred in this same group of pa
tients. The wide variety of pathogens involved in these opportunistic infec tions and the severity of the illness have suggested a compromised state w ith little or no normal defenses, m uch like that found in immunosuppressed patients. This m u lti faceted disease complex of oppor tunistic infection and Kaposi’s sar coma has been termed an Acquired Immunodeficiency Syndrome (AIDS). The factors re sp o n sib le for the immunosuppression and for devel opment of Kaposi’s sarcoma are not known. Many potential agents have been suggested. Cytomegalovirus in fection has been a common finding. CMV is shed in semen and saliva and has been shown to cause im m uno suppression in mice and human be ings.16,17 CMV has been cultured from tumor tissue, and Kaposi’s sarcoma has been linked with CMV,1214 so the virus is a candidate as the etiologic fac tor or at least one of the factors of Kaposi’s sarcoma. Cytomegalic inclu sions are most prominent in vascular e n dothelial cells in a d u lts 17 and Kaposi’s sarcoma may be derived from endothelial cells. The use of drugs has also been suggested as a possible contributing factor in the development of Kaposi’s sarcoma. Volatile nitrites may have some role in the development of the disease. Both amyl nitrite and isobutyl nitrite are commonly used (more than 86%) as sexual stimulants or recre ational drugs by homosexual men.18 Many of the heterosexual patients with Kaposi’s sarcoma also have a history of drug use. Other factors associated with h o m o s e x u a l p ra c tic e s in c lu d e mucosal exposure to seminal plasma and the use of steroid creams, both of w h ic h have im m unosuppressive properties.19 21 The patients have had profound defects in the cell-mediated immune response. All were anergic, had T-lymphocyte depletion, and had markedly depressed lymphoproliferative responses. It is im portant that dentists are aware of this increase in Kaposi’s sar coma because the disease may first occur as an oral lesion. Also, oral can didiasis, lymphadenopathy, fever, weight loss, and diarrhea may be pres ent for several months as prodromal signs and symptoms in patients with AIDS. Because Kaposi’s sarcoma is generally considered to be a disease that appears in the skin, it may not be
D IA G N O SIN G
considered in the early differential diagnosis when the first lesion appears in the oral cavity. This early oral lesion may resemble a pyogenic granuloma and may be diagnosed as such.22 Oral lesions with a vascular component should be considered as potentially dangerous until the history and histo pathologic findings prove otherwise.
Summary A brief report on Kaposi’s sarcoma as encountered in Africa and in a small segment of older patients in this and other countries is presented. The re cent outbreak over the past two years of Kaposi’s sarcoma in patients with an acquired im m unodeficiency syn drome is reviewed. Because of the in creased incidence of this disease, den tists must be familiar with the oral manifestations.
The publication of this series is coordinated by - the Veterans Administration Dental Education Center I West Coast and supported in part by funding provided by the Veterans Administration Exchange of Medical Information Program. The opinions and assertions contained herein are those of the authors and are not to be construed as official or necessarily representing the views of the Veterans Administration.
Dr. Wescott is director, Veterans Administra tion Dental Education Center, and professor, de partm ent of oral diagnosis, radiology, and pathology, Loma Linda University School of Den tistry. Dr. Correll is associate director, Veterans Administration Dental Education Center,. Los Angeles, and clinical assistant professor, depart ment of pathology, University of Southern Cali fornia School of Dentistry, Los Angeles. Address request for reprints to Dental Education Center, (161), Veterans Administration Medical Center, West Los Angeles, 90073. 1. Kaposi, M. Idiopathic multiple pigmented sarcoma of the skin. Arch Dermatol 4:265-273, 1872. 2. Mansell, P.W. Kaposi’s sarcoma— an emerg ing epidemic. Cancer Bull 34:72-74,1982. 3. Montgomery, H. Dermatopathology. New York, Harper & Row, 1967, p 1111. 4. Hajdu, S.I. Pathology of soft tissue tumors. Philadelphia, Lea & Febiger, 1979, p 401. 5. Ulbright, T.M., and Santa Cruz, D.J. Kaposi's sarcoma: relationship with hematologic, lym phoid, and thymic neoplasia. Cancer 47:963-973, 1981. 6. Reidy, T.J., and others. Kaposi’s sarcoma and immune hemolysis. Cancer 50:1297-1298, 1982. 7. Harwood, A.R., and others. Kaposi’s sar coma in recipients of renal transplants. Amer J Med 67:759-765, 1979. 8. Farman, A.G., and Uys, P.B. Oral Kaposi’s sarcoma. Oral Surg 39:288-296, 1975. 9. Haim, S., and others. Kaposi’s sarcoma in as sociation with immunosuppressive therapy. Isr J Med Sci 8:1993-1997, 1972. 10. Safai, B., and Good, R.A. Kaposi’s sarcoma: a review and recent developments. CA 3:2-12, 1981. 11. Giraldo, G.; Beth, E.; and Haguenau, F. Herpes-type virus particles in tissue culture of Kaposi’s sarcoma from different geographic re gions. J Natl Cancer Inst 49:1509-1526, 1972. 12. Giraldo, G.; Beth, E.; and Henle, W. Anti
ORAL DISEA SE
body patterns to herpes viruses in Kaposi’s sar coma: serological association of European Kaposi’s sarcoma with cytomegalovirus. Int J Cancer 15:839-848,1975. 13. Giraldo, G.; Beth, E.; and Henle, W. Anti body patterns to herpes viruses in Kaposi’s sar coma II. Serological association of American Kaposi’s sarcoma with cytomegalovirus. Int J Cancer 22:126-131, 1978. 14. Boldogh, I., and others. Kaposi’s sarcoma IV Detection of CMV DNA and CMV RNA and CMNA in tumor biopsies. IntJ Cancer 28:469-474, 1981. 15. Durack, D.T. Opportunistic infections and Kaposi’s sarcoma in homosexual men. N Eng J Med 305:1465-1467, 1981. 16. Hamilton, J.R.; Overall, J.C., Jr.; and Glas gow, L.A. Synergistic effect on mortality in mice with murine cytomegalovirus and Pseudomonas aeruginosa, StaphyJococcus aureus, or Candida albicans infections. Infect Im m un 14:982-989, 1976. 17. Carney, W.P., and others. Analysis of T ly m p h o c y te subsets in c y to m e g a lo v iru s mononucleosis. J Immunol 126:2114-2116,1981. 18. Wong, T.W., and Warner, N.E. Cytomegalic inclusion disease in adults. Arch Pathol 74:403422,1962. 19. Centers for Disease Control Task Force Re port: epidemiologic aspects of the current out break of Kaposi’s sarcoma and opportunistic in fections. N Eng J Med 306:248-252,1982. 20. Lord, E.M.; Sensabaugh, G.F.; and Stites, D.P. Immunosuppressive activity of hum an sem inal plasma. J Immunol 118:1704-1711,1977. 21. Anderson, D.J., and Tarter, T.H. Immuno suppressive effects of mouse seminal plasma components in vivo and in vitro. J Im m unol 128:535-539, 1982. 22. Neumann, H.H. Use of steroid creams as a p o s s ib le cause o f im m u n o s u p r e s s io n in homosexuals (letter). N Eng J Med 306:935,1982. 23. Breauer, M.K.; Gates, P.E.; and Doyle, J.L. Visceral Kaposi’s sarcoma presenting w ith gingi val lesions. Oral Surg 50:151-155,1980.
W escott-Correll: NODULAR M ASS IN H ARD PALATE ■ 1043