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Primary Sarcoidosis of the Scrotum: Case Report
JOUR.'.'.[A.L O.F' UROLOGY
_August 1961 Hltil by The Williams & Wilkins Co. Printed in., U.S.A.
PRIJ\IARY SARCOIDOSIS OF THE: SCROTUM· CASE REPORT K. F. HAUSFELD Departrnent of Urologg, The Children's Hospital, Akron, Ohio
Sareoidosis or Boeck's sa.rcoi
clinicopathologic review· of cases, including 22 autopsieo. Am. ,J. Clin. Path., 19: 725--74D, 194P. '' Longcope, W. T. and :Freiman, D. G.: A study of sarcoidosis based on a combined investigation of mo eases including 30 autopsies from Johns Hopkins Hospital, and Massachtrnetts General Hospit.a.1. Medicine, 31: 1--132, 1D52. 5 .F1·eiman, D. G.: Medical progress: Sarcoidosis, Kew Eng. J. MecL, 239: 6CH67L 709-716, 743-740, Hl48.
7 .Jaques, ·w. E.· Sa.rcoidosis. Arch. P:ith., 53: 558-502, Hl52. 8 Kraus, L, · C:cnital sa.rcoidosis: Ca.se report
and review of the litern.t.me. J. "Crol., 80: 367--370, l9.5S 2139
coidosis have been reported in the world lit.em. ture. According to J\Tc:Govcrn and [13 cases of sarcoidosis ha.ve been found in children aged 1.5 years or younger. primary genital sarcoiclosis in a whii,e boy is extremely rare and \\·orthy of a report. CASE Rt<:POH.T
A 5-year-old white boy was first seen on November 6, 1958. He complained of ~welling ot the scrotum and stated that on October 1958, while voiding in his yard, a bug had bitten his exposed scrotum. Thm·r;aft<)r, swelling in the area. of the bug bite wa.s note
270
K. F. HAUSFELD
of 10 tuberculin units was positive; whereas a similar test for histoplasmin was negative. An intravenous urogram showed prompt and good quantitative excretion with hypaque; normal pelviocalyceal anatomy; and no ureteral displacement. A roentgen examination of the chest in posteroanterior and lateral views showed the cardiac silhouette to be normal in size and contour. The lung fields showed no evidence of infiltration. There was a fullness of the mediastinum which was attributed to vascular densities or possibly some involution of the thymus or enlarged mediastinal lymph nodes. The diaphragms and bony thorax showed no abnormality. When admitted to the hospital, the patient had febrile elevations to 100.4F of a noncharacteristic type but all temperature, pulse, and respiratory excursions became normal within a few days after surgery. On November 21, 1958 the scrotal mass was excised. It was found to extend through the scrotal layers to the tunica vaginalis and was
adherent to the tunica albuginea of the left testis at the lower pole. Three small lymph nodes were also removed from the left inguinal area. Convalescence from the procedure was uneventful and the patient was discharged from the hospital on December 3, 1958. One week after the scrotal lesion had been excised, roentgen examination of the chest disclosed that the superior mediastinal fullness no longer was present, and no pulmonary infiltration was present. The gross specimen was an irregularly shaped piece of tissue weighing 14.5 gm. On one surface was a piece of skin measuring 2. 7 by 2.0 cm., in the center of which was a purplish, discolored area 16 mm. in diameter. The mass was firm in consistency and appeared to be completely surrounded by normal appearing connective tissue. A suture was placed at one point to mark the area dissected free from the testicle. On cut section the tumor mass had a homogeneous, yellowish-gray appearance. The specimen also consisted of three small lymph nodes measuring
Fm. 1. Photomicrographs, XlOO. A, primary tumor showing tubercle formation. B, inguinal lymph node.
271
SARCOIDOSIS OF SCROTUM
5 to l O mm. in diameter. A culture of one node was made for tubercle bacilli and fungi. Grossly the nodes were not remarkable. Microscopic description (9 sections H & E): Senn of the nine sections stained with hematoxylin and eosin were from the cutaneous tumor mass and an eighth section was from the attachment to the testicular infundilmlum. The sections from tbe main tumor mass disclosed that it lay in the subcutaneous tissue and extended up to the deeper layers of the dermis. _1.Jl sections of the tumor were essentially similar in appearance (fig. l, A). They consisted of numerous areas of epithelioid proliferation without central necrosis, caseation or hyaline degeneration. These discrete tubercles were separatc>d by considerable lymphoid tissue and a rather dense connective tissue stroma. Large nrnltinuckated giant cells were numerous and wern usually found within the proliferated epitheloid centers of tbe tubercles. Such giant cells were seen to contain clear, hyaline cytoplasmic bodies, as are observed in sarcoid. There was no eosinophilic cell infiltration throughout the sectionF. The section taken from the infundibulum c011sisted of Yascular connective tissLie and was free of tumor involvement except for one very small perivascular area of lymµhocytic cell invoh·ement, in the center of which there was obserYcd an early epithelioid cell proliferation. The remaining hematoxylin and cosin stained section was from the inguinal nodes (fig. 1, B). fn this section the lymphoid architecture was fairly well preserl'ed with secondary follicle formation persisting in the subcapsular zone. Throughout the howeyer, there were rmrnerous small tubercle formations with cpithelioicl centers. These centers were slightly different in appearance than those seen in the subcutaneous mass in that they appeared denser and free of necrosis, caseation and hyalinization. Careful examination of the sections stained with acid fast and Gram stains failed to reveal any etiologic sueh as protozoan fungi or bacteria. Diagnosis: Sarcoid. On .January 17, 1960, the patient was readmitted to the hospital for further study rmd evaluation. .A repeated routine urinalysis disclosed a pH. of 6, no albumin or sugar and an occasional
leukocyte per high pmvcr field microscopically Three stool examinations were negative for on\. parasites and occult blood. Hematological and chemical examinations o!: the blood were as follmrn: erythrocytes 4, hemoglobin 12.5 gm.; leukocytes 4,400, witL lymphocytes 49 per cent, monocytes 4 per polymorphonuclear leukoeytes 44 per cent and immature polymorphonuclear leukocytes B per cent on differential examination; blood sugar lH.1 mg. per cent; urea nitrogen 11 mg. per cent., creatinine 1.2 mg. per cent; cakium 10.3 mg per cent; inorganic phosphorus 4.5 mg. per c:en!; total protein 5.0 mg. per cent with albumin and globulin 1.G or an albumin-globulin ratio of 2 · 1; urea clearance 100 11er cent. A tuberculin skin test utilizing P.P.D. of 10 tuberculin units was positive. The chest film. showed no evidenc:e of a logical process involving the heart or There was no evidence of racliol.ogicol lympli node enlargement An intravenous urogram revealed nomrn.l renal morphology, no displace1rn,nt of eitlwt kidney or ureter and normal excretion of During this hospital stay tbe patient's temperature, pulse and respirations 1yere normal. DISCUSSION
Tbe etiology of sarcoidosis remains oliscnre. l'lfany etiological agents have been as causes of this disease, but most of these agnnti: have pertained to tubercL1losis. 9 · io. H This .is justifiable inasmuch as 10 per cent to 25 per ccni. of sarcoiclosis cases develop active tuber, culosis. 6 • 12 Hmycver, other etiological agent,~ mentioned in the causation of this disease irn:lnd 0, Brucclla, 13 l\fycobacteriurn leprwc, 14 Trcponc•rrw pallidum, 15 protozoa, 16 • 17 fungi,"· 19 9 Pinner, M.: N oncaseating tuberculosis: sis of literat1ne. Am. Rev. Tuberc, 37:
1938. 10
Pautrier, L. M.: Sarcoidosis. Brit . .J. Tube re ..
42: 1-17, l\J48. u Carnes, W. H. R.ncl Raff el, 8. · Comparison sarcoiclosis and tuberculosis \\"ith respect to complement fixation with antigens derived from tubercle bacillus. Bull ,fohlls Hopki1rn 1-lo~J.>.,
85: 204-220. 19'19.
12 Riley, ·E. A.· Boeck's sarcoid; review bn,ed 1.1pon clinical study of 52 cases. Am. Rev. Tnberc ..
62: 231-285, 1950. 13 Harrell, G. T.: Genernlizecl rnrcoidosis. Bowman Grey School Med., 1: 1-4, 194;1 . 14 Rabello, ,Jr.: Dom1ees nollvelles f.H.lt1r ]'interpretation de ]'affection de Besnicr-Boeck:
272
K. F. HAUSFELD
and foreign bodies, especially silicon containing material. 21 The possibility that the disease represents a neoplastic process has also been considered. 21 Recently, Nematode larvae22 and allergy to pine needles23 have been incriminated as causing the disease. role de la lepre. Ann. de dermat. et syph., 7: 571-597, '36.
15 Frazier, C. N. and Hu, C. K.: Isolation of Treponema ·pallidum from subcutaneous sarcoid. Proc. Soc. Exper. Biol. and Med., 30: 898-901,
1933.
16 Stone, C. T., Jr.: Pathology of Boeck's sarcoid. New Orleans M. and S. J., 98: 369-373, 1946. 17 Tice, F. and Sweany, H. C.: Fatal case of Besnier-Boeck-Schaumann's disease with autopsy findings. Ann. Int. Med., 15: 597-609, 1941. 18 Harrell, G. T.: Generalized sarcoidosis of Boeck: Clinical review of 11 cases with studies of blood and etiological factors. Arch. Int. Med.,
65: 1003-1034, 1940. 19 Higgins, H. L.: Pulmonary sarcoidosis. Connecticut M. J., 11: 330-339, 1947.
Refvem, 0.: Chronic granulomas in alimentary tract caused by minute mineral particles: "Boeck's disease" and occurrence of minute mineral particles (preliminary report). Acta path. microbial. scandinav., 25: 107-121, 1948. 21 Scott, R. B.: Sarcoidosis of Boeck. Brit. Med. J., 2: 777-781, 1938. 22 Jaques, W. E.: Relationship of Nematode larvae to generalized sarcoidosis. Arch. Path.,
If one is able to believe the statement, substantiated by the mother of the 5-year-old boy, that his disease process started from a bug bite on the scrotum, then one must consider a vector in transmission of sarcoidosis, because at no time has the boy been away from his home in Ohio and he has not been found to suffer from active disease processes thought to produce sarcoidosis. No specific therapy was administered as it is generally assumed that corticosteroids should be used only when necessary to produce a clinical remission of the disease. 1 The rapid subsidence of the mediastinal enlargement present on the initial roentgen examination of the chest is subject to discussion. SUMMARY
20
53: 550-557, 1952.
23 Cummings, M. M. and Hudgins, P. C.: Chemical constituents of pine pollen and their possible relationship to sarcoidosis. Am. J. Med. Sc.,
236: 311-317, 1958.
A white 5-year-old boy was found to have primary sarcoidosis of the scrotum with secondary lymph node involvement. The onset of the disease was attributed to a bug bite on the scrotum. Following removal of the lesion he has remained asymptomatic. Etiologic agents producing the disease are briefly discussed. ?S6 Second National Bldg., Akron, Ohio
_August 1961 Hltil by The Williams & Wilkins Co. Printed in., U.S.A.
PRIJ\IARY SARCOIDOSIS OF THE: SCROTUM· CASE REPORT K. F. HAUSFELD Departrnent of Urologg, The Children's Hospital, Akron, Ohio
Sareoidosis or Boeck's sa.rcoi
clinicopathologic review· of cases, including 22 autopsieo. Am. ,J. Clin. Path., 19: 725--74D, 194P. '' Longcope, W. T. and :Freiman, D. G.: A study of sarcoidosis based on a combined investigation of mo eases including 30 autopsies from Johns Hopkins Hospital, and Massachtrnetts General Hospit.a.1. Medicine, 31: 1--132, 1D52. 5 .F1·eiman, D. G.: Medical progress: Sarcoidosis, Kew Eng. J. MecL, 239: 6CH67L 709-716, 743-740, Hl48.
7 .Jaques, ·w. E.· Sa.rcoidosis. Arch. P:ith., 53: 558-502, Hl52. 8 Kraus, L, · C:cnital sa.rcoidosis: Ca.se report
and review of the litern.t.me. J. "Crol., 80: 367--370, l9.5S 2139
coidosis have been reported in the world lit.em. ture. According to J\Tc:Govcrn and [13 cases of sarcoidosis ha.ve been found in children aged 1.5 years or younger. primary genital sarcoiclosis in a whii,e boy is extremely rare and \\·orthy of a report. CASE Rt<:POH.T
A 5-year-old white boy was first seen on November 6, 1958. He complained of ~welling ot the scrotum and stated that on October 1958, while voiding in his yard, a bug had bitten his exposed scrotum. Thm·r;aft<)r, swelling in the area. of the bug bite wa.s note
270
K. F. HAUSFELD
of 10 tuberculin units was positive; whereas a similar test for histoplasmin was negative. An intravenous urogram showed prompt and good quantitative excretion with hypaque; normal pelviocalyceal anatomy; and no ureteral displacement. A roentgen examination of the chest in posteroanterior and lateral views showed the cardiac silhouette to be normal in size and contour. The lung fields showed no evidence of infiltration. There was a fullness of the mediastinum which was attributed to vascular densities or possibly some involution of the thymus or enlarged mediastinal lymph nodes. The diaphragms and bony thorax showed no abnormality. When admitted to the hospital, the patient had febrile elevations to 100.4F of a noncharacteristic type but all temperature, pulse, and respiratory excursions became normal within a few days after surgery. On November 21, 1958 the scrotal mass was excised. It was found to extend through the scrotal layers to the tunica vaginalis and was
adherent to the tunica albuginea of the left testis at the lower pole. Three small lymph nodes were also removed from the left inguinal area. Convalescence from the procedure was uneventful and the patient was discharged from the hospital on December 3, 1958. One week after the scrotal lesion had been excised, roentgen examination of the chest disclosed that the superior mediastinal fullness no longer was present, and no pulmonary infiltration was present. The gross specimen was an irregularly shaped piece of tissue weighing 14.5 gm. On one surface was a piece of skin measuring 2. 7 by 2.0 cm., in the center of which was a purplish, discolored area 16 mm. in diameter. The mass was firm in consistency and appeared to be completely surrounded by normal appearing connective tissue. A suture was placed at one point to mark the area dissected free from the testicle. On cut section the tumor mass had a homogeneous, yellowish-gray appearance. The specimen also consisted of three small lymph nodes measuring
Fm. 1. Photomicrographs, XlOO. A, primary tumor showing tubercle formation. B, inguinal lymph node.
271
SARCOIDOSIS OF SCROTUM
5 to l O mm. in diameter. A culture of one node was made for tubercle bacilli and fungi. Grossly the nodes were not remarkable. Microscopic description (9 sections H & E): Senn of the nine sections stained with hematoxylin and eosin were from the cutaneous tumor mass and an eighth section was from the attachment to the testicular infundilmlum. The sections from tbe main tumor mass disclosed that it lay in the subcutaneous tissue and extended up to the deeper layers of the dermis. _1.Jl sections of the tumor were essentially similar in appearance (fig. l, A). They consisted of numerous areas of epithelioid proliferation without central necrosis, caseation or hyaline degeneration. These discrete tubercles were separatc>d by considerable lymphoid tissue and a rather dense connective tissue stroma. Large nrnltinuckated giant cells were numerous and wern usually found within the proliferated epitheloid centers of tbe tubercles. Such giant cells were seen to contain clear, hyaline cytoplasmic bodies, as are observed in sarcoid. There was no eosinophilic cell infiltration throughout the sectionF. The section taken from the infundibulum c011sisted of Yascular connective tissLie and was free of tumor involvement except for one very small perivascular area of lymµhocytic cell invoh·ement, in the center of which there was obserYcd an early epithelioid cell proliferation. The remaining hematoxylin and cosin stained section was from the inguinal nodes (fig. 1, B). fn this section the lymphoid architecture was fairly well preserl'ed with secondary follicle formation persisting in the subcapsular zone. Throughout the howeyer, there were rmrnerous small tubercle formations with cpithelioicl centers. These centers were slightly different in appearance than those seen in the subcutaneous mass in that they appeared denser and free of necrosis, caseation and hyalinization. Careful examination of the sections stained with acid fast and Gram stains failed to reveal any etiologic sueh as protozoan fungi or bacteria. Diagnosis: Sarcoid. On .January 17, 1960, the patient was readmitted to the hospital for further study rmd evaluation. .A repeated routine urinalysis disclosed a pH. of 6, no albumin or sugar and an occasional
leukocyte per high pmvcr field microscopically Three stool examinations were negative for on\. parasites and occult blood. Hematological and chemical examinations o!: the blood were as follmrn: erythrocytes 4, hemoglobin 12.5 gm.; leukocytes 4,400, witL lymphocytes 49 per cent, monocytes 4 per polymorphonuclear leukoeytes 44 per cent and immature polymorphonuclear leukocytes B per cent on differential examination; blood sugar lH.1 mg. per cent; urea nitrogen 11 mg. per cent., creatinine 1.2 mg. per cent; cakium 10.3 mg per cent; inorganic phosphorus 4.5 mg. per c:en!; total protein 5.0 mg. per cent with albumin and globulin 1.G or an albumin-globulin ratio of 2 · 1; urea clearance 100 11er cent. A tuberculin skin test utilizing P.P.D. of 10 tuberculin units was positive. The chest film. showed no evidenc:e of a logical process involving the heart or There was no evidence of racliol.ogicol lympli node enlargement An intravenous urogram revealed nomrn.l renal morphology, no displace1rn,nt of eitlwt kidney or ureter and normal excretion of During this hospital stay tbe patient's temperature, pulse and respirations 1yere normal. DISCUSSION
Tbe etiology of sarcoidosis remains oliscnre. l'lfany etiological agents have been as causes of this disease, but most of these agnnti: have pertained to tubercL1losis. 9 · io. H This .is justifiable inasmuch as 10 per cent to 25 per ccni. of sarcoiclosis cases develop active tuber, culosis. 6 • 12 Hmycver, other etiological agent,~ mentioned in the causation of this disease irn:lnd 0, Brucclla, 13 l\fycobacteriurn leprwc, 14 Trcponc•rrw pallidum, 15 protozoa, 16 • 17 fungi,"· 19 9 Pinner, M.: N oncaseating tuberculosis: sis of literat1ne. Am. Rev. Tuberc, 37:
1938. 10
Pautrier, L. M.: Sarcoidosis. Brit . .J. Tube re ..
42: 1-17, l\J48. u Carnes, W. H. R.ncl Raff el, 8. · Comparison sarcoiclosis and tuberculosis \\"ith respect to complement fixation with antigens derived from tubercle bacillus. Bull ,fohlls Hopki1rn 1-lo~J.>.,
85: 204-220. 19'19.
12 Riley, ·E. A.· Boeck's sarcoid; review bn,ed 1.1pon clinical study of 52 cases. Am. Rev. Tnberc ..
62: 231-285, 1950. 13 Harrell, G. T.: Genernlizecl rnrcoidosis. Bowman Grey School Med., 1: 1-4, 194;1 . 14 Rabello, ,Jr.: Dom1ees nollvelles f.H.lt1r ]'interpretation de ]'affection de Besnicr-Boeck:
272
K. F. HAUSFELD
and foreign bodies, especially silicon containing material. 21 The possibility that the disease represents a neoplastic process has also been considered. 21 Recently, Nematode larvae22 and allergy to pine needles23 have been incriminated as causing the disease. role de la lepre. Ann. de dermat. et syph., 7: 571-597, '36.
15 Frazier, C. N. and Hu, C. K.: Isolation of Treponema ·pallidum from subcutaneous sarcoid. Proc. Soc. Exper. Biol. and Med., 30: 898-901,
1933.
16 Stone, C. T., Jr.: Pathology of Boeck's sarcoid. New Orleans M. and S. J., 98: 369-373, 1946. 17 Tice, F. and Sweany, H. C.: Fatal case of Besnier-Boeck-Schaumann's disease with autopsy findings. Ann. Int. Med., 15: 597-609, 1941. 18 Harrell, G. T.: Generalized sarcoidosis of Boeck: Clinical review of 11 cases with studies of blood and etiological factors. Arch. Int. Med.,
65: 1003-1034, 1940. 19 Higgins, H. L.: Pulmonary sarcoidosis. Connecticut M. J., 11: 330-339, 1947.
Refvem, 0.: Chronic granulomas in alimentary tract caused by minute mineral particles: "Boeck's disease" and occurrence of minute mineral particles (preliminary report). Acta path. microbial. scandinav., 25: 107-121, 1948. 21 Scott, R. B.: Sarcoidosis of Boeck. Brit. Med. J., 2: 777-781, 1938. 22 Jaques, W. E.: Relationship of Nematode larvae to generalized sarcoidosis. Arch. Path.,
If one is able to believe the statement, substantiated by the mother of the 5-year-old boy, that his disease process started from a bug bite on the scrotum, then one must consider a vector in transmission of sarcoidosis, because at no time has the boy been away from his home in Ohio and he has not been found to suffer from active disease processes thought to produce sarcoidosis. No specific therapy was administered as it is generally assumed that corticosteroids should be used only when necessary to produce a clinical remission of the disease. 1 The rapid subsidence of the mediastinal enlargement present on the initial roentgen examination of the chest is subject to discussion. SUMMARY
20
53: 550-557, 1952.
23 Cummings, M. M. and Hudgins, P. C.: Chemical constituents of pine pollen and their possible relationship to sarcoidosis. Am. J. Med. Sc.,
236: 311-317, 1958.
A white 5-year-old boy was found to have primary sarcoidosis of the scrotum with secondary lymph node involvement. The onset of the disease was attributed to a bug bite on the scrotum. Following removal of the lesion he has remained asymptomatic. Etiologic agents producing the disease are briefly discussed. ?S6 Second National Bldg., Akron, Ohio