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Case report: The ultrasound appearances of testicular microlithiasis (‘Snow Storm’ testis): A case complicated by testicular seminoma
ClinicalRadiology(1993) 47, 65-67
Case Report: The Ultrasound Appearances of Testicular Microlithiasis ('Snow Storm' Testis): A Case Complicated by Testicular Seminoma I. S. D. R O B E R T S a n d C. F. L O U G H R A N *
Departments of Pathology and *Radiology, Macclesfield District General Hospital, MacclesfieM A case is described in which a patient with bilateral testicular seminomas was found also to have testicular microlithiasis. The ultrasound and pathological features are described. R o b e r t s , I.S.D. & L o u g h r a n , C . F . (1993). ClinicalRadiology 47, 65-67. Case R e p o r t : T h e U l t r a s o u n d A p p e a r a n c e s o f Testicular Microlithiasis ( ' S n o w S t o r m ' Testis): A Case C o m p l i c a t e d by Testicular Seminoma
A t u l t r a s o u n d the n o r m a l testis shows h o m o g e n e o u s m e d i u m level reflectivity which presents an excellent b a c k g r o u n d for the detection o f testicular t u m o u r s . I n the m a j o r i t y o f such cases, the t u m o u r is localized and usually presents as an a r e a o f either reduced o r m i x e d reflectivity. W e wish to r e p o r t a case o f a p a t i e n t with b i l a t e r a l s e m i n o m a s in which this typical p a t t e r n was superimposed u p o n w i d e s p r e a d highly reflective foci. This p a t t e r n has previously been described in testicular microlithiasis, a rare c o n d i t i o n in which calcified deposits are laid d o w n in a t r o p h i c seminiferous tubules. W e believe this is the first r e p o r t o f the two c o n d i t i o n s occurring in the same patient a n d wish to d r a w a t t e n t i o n to the u n u s u a l u l t r a s o u n d a p p e a r a n c e s which c h a r a c t e r i z e d the case.
CASE REPORT A 26-year-old male presented with a 6 month history of a swollen left testis. There was no past history of cryptorchidism and on examination both testes were intrascrotal. The left testis measured 8 cm in diameter and was hard. An ultrasound study of the scrotum was performed. This confirmed enlargement of the testis which had a diffuse abnormal pattern, consisting of hypoechoic nodules 0.5 1.0 cm in size. The dominant feature, however, was the presence of widespread highly reflectivefoci distributed throughout the testicular parenchyma (Fig. 1). Two larger but similar hypoechoic nodules, measuring approximately
1.5 cm in diameter, were shown in the lower pole of the right testis, but again the dominant feature was the presence of widespread highly reflective foci (Fig. 2). Alpha feto protein levels (< 5 ng/ml on two occasions) were normal as were serial human chorionic gonadotrophin (HCG) estimations (<5 i.u./l). The chest X-ray was normal. He subsequently underwent bilateral orchidectomy.
PATHOLOGY T h e left testis weighed 154 g a n d m e a s u r e d 9 x 6 x 4 cm in size. I t was t o t a l l y r e p l a c e d b y s e m i n o m a (Fig. 3). H i s t o l o g i c a l l y , foci o f calcification were s h o w n t h r o u g h o u t the testis, o c c u r r i n g m a i n l y within the c o l l a g e n o u s t u m o u r stroma. E a c h calcific focus was spherical a n d m e a s u r e d a b o u t 300 /~m in d i a m e t e r . T h e testis was r a d i o g r a p h e d following r e m o v a l a n d this c o n f i r m e d the w i d e s p r e a d n a t u r e o f the calcific foci (Fig. 4). The right testis weighed 53 g a n d m e a s u r e d 5.5 • 3.5 x 3 c m in size. T h e t u m o u r was localized to the lower p o l e a n d m e a s u r e d 3 x 2 x 1 c m in size. Histologically, this was also semin o m a a n d c o n t a i n e d similar calcific foci. Sections o f nont u m o r o u s testis d e m o n s t r a t e d w i d e s p r e a d calcified intrat u b u l a r structures identical in a p p e a r a n c e to those within the tumQur (Fig. 5). The tubules s h o w e d v a r y i n g degrees o f a t r o p h y which was m o s t m a r k e d in those c o n t a i n i n g calcific deposits. A small p r o p o r t i o n o f tubules c o n t a i n e d m a t u r e s p e r m a t o z o a . N o element o f i n t r a t u b u l a r carci-
Fig. 1 - Left testis. Multiple small (2-3 mm) highly reflective foci are shown throughout the testicular parenchyma. Correspondence to: Dr C. F. Loughran, Department of Radiology, Macclesfield District General Hospital, Victoria Road, Macclesfield, Cheshire.
Fig; 2 - Right testis. Again multiple bright foci are shown throughout the testis. Note the presence of tumour nodules in the lower pole of the testis.
66
CLINICAL RADIOLOGY
DISCUSSION
Fig. 3 - Resected specimen (left testis). The cut surface reveals the testis to be almost entirely replaced by seminoma.
Fig. 4 - Radiograph of the excised left testis. Note the widespread calcific deposits throughout the testicular parenchyma.
7t
v
Fig. 5 - Atrophic seminiferous tubules containing calcified deposits.
noma was identified. Both tumours showed multiple small and large areas of necrosis, seen as pale areas in the gross specimen (Fig. 3). No teratomatous elements were noted in either tumour. He is now under regular review and currently remains well with no evidence of tumour recurrence or spread.
Ultrasound of the scrotum is an accurate and safe means of evaluating intrascrotal pathology. Use of transducers with high frequency probes of 7.5-10 MHz enables detailed anatomic depiction of testicular structure and disease. This improves diagnostic a c c u r a c y 25% of tumours are incorrectly diagnosed at the initial physical examination. In assessing testicular tumours ultrasound has proven reliable. It clarifies the nature of a palpable mass and helps determine whether it is intra- or extra-testicular. Reports indicate that in terms of sensitivity and specificity accuracy rates in excess of 95% are possible in the best hands [1]. Tumours of the testis are characteristically welldefined, predominantly hypo-reflective lesions. Generally, it is not possible to distinguish cell types on the basis of the ultrasound appearance, although seminomas usually have a more uniform pattern whereas teratomas tend to be more disorganized with foci o f high reflectivity within them. In analysing the sonographic changes noted in 29 patients with testicular neoplasia Grantham et al. [2] considered three factors of significance when diagnosing tumour by ultrasound: mass, bright echogenic foci and diffuse parenchymal testicular change. Of interest is the observation that bright echogenic foci were visible in 19 (66%) of their patients. Four patients had ultrasoundguided biopsies of these foci; one was due to a focus of cartilage and bone in a malignant teratoma, another due to a calcific focus in a mature teratoma and two in areas of non-calcific scarring in association with regressed germ cell tumours. In the case described, the most prominent characteristic was the widespread echogenic speckling. The underlying pathological cause for the speckling was the deposition o f calcium in the testicular parenchyma. Histological examination demonstrated that the calcifications had developed within atrophic seminiferous tubules and these had subsequently become incorporated within the tumour. Diffuse intratubular calcification as seen here has been termed testicular microlithiasis. This is a rare condition, characterized by the presence throughout the testis of innumerable tiny deposits of calcium within degenerating tubular cells. Cases have been noted in associated with cryptorchidism, Klinefelter's syndrome and male pseudohermaphroditism [3,4]. It has also been described in a patient with pulmonary alveolar microlithiasis and has been noted in normal scrotal testes [5]. Testicular microlithiasis has been previously described at scrotal ultrasound. Characteristically, as in this case, many highly reflective deposits were shown distributed throughout the testicular parenchyma [5,6]. Focal intratubular calcification has been previously recognized histologically around testicular tumours [7]. Kragel et al. [8] recently reported a case of intratubular germ cell neoplasia associated with numerous intratubular microliths but to our knowledge this is the first report of a testicular tumour being associated with the typical sonographic features of testicular microlithiasis. Bilateral testicular seminoma is rare, with a frequency of less than 1% of testicular tumours [9]. Synchronous tumours are even less common, accounting for about one third of bilateral tumours. A further unusual feature of this case is the young age of the patient at presentation, seminomas typically occurring towards the end o f the fourth decade. The aetiology of testicular tumours is not
ULTRASOUND APPEARANCES OF TESTICULAR MICROL1THIASIS
certain but many cases are associated with gonadal dysgenesis as occurs in cryptorchid testes. The presence of bilateral tumours and young age of the patient in this case suggests the possibility of an intrinsic testicular abnormality predisposing to tumour development. The aetiology ofintratubular microliths is also obscure. It has been suggested that they reflect testicular immaturity [10]. In this case it is tempting to speculate that testicular microlithiasis predated the development of tumour and predisposed the patient to it. Whatever the relationship of microlithiasis to tumour, caution should be taken when interpreting such sonographic appearances; an underlying neoplasm should be carefully excluded and follow-up ultrasound screening should be considered. REFERENCES 1 Fowler RC, Chennels PM, Ewing R. Scrotal ultrasonography: a clinical evaluation. British Journal of Radiology 1987;60:649-654.
67
2 Grantham JG, Charboneau JW, James EM, Kirschling RJ, Kvols LK, Segura JW, Wold LE. Testicular neoplasms: 29 tumours studied by high resolution ultrasound. Radiology 1985; 157:775-780. 3 Bieger RC, Passarge E, McAdams AJ. Testicular intratubular bodies. Journal of Clinical Endocrinology 1965;25:1340-1346. 4 Nistal M, Paniagua R, Diez-Pardo JA. Testicular microlithiasis in two children with bilateral cryptorchidism. Journal of Urology 1979;121:535-537. 5 Jaramillo D, Perez-Atayde A, Teele RL. Sonography of testicular microlithiasis. Urologic Radiology 1989; 11:55 57. 6 Doherty F J, Mullins TL, Sant GR, Drinkwater MA, Ucci AA. Testicular microlithiasis. Journal of Ultrasound in Medicine 1987;6:389-392. 7 Azzopardi JG, Mostofi FK, Theiss EA. Lesions of testes observed in certain patients with widespread choriocarcinoma and related tumours. American Journal of Pathology 1961;38:207-219. 8 Kragel PJ, Delvecchio D, Orlando R, Garvin DF. Ultrasonographic findings of testicular microlithiasis associated with intratubular germ cell neoplasia. Urology 1991;37:66-68. 9 Mostofi FK, Price EB. Tumours of the male genital system. Armed Forces Institute of Pathology, 2nd series, 1973. 10 Priebe CJ, Garret R. Testicular calcification in a 4-year old boy. Paediatrics 1970;46:785-788.
Case Report: The Ultrasound Appearances of Testicular Microlithiasis ('Snow Storm' Testis): A Case Complicated by Testicular Seminoma I. S. D. R O B E R T S a n d C. F. L O U G H R A N *
Departments of Pathology and *Radiology, Macclesfield District General Hospital, MacclesfieM A case is described in which a patient with bilateral testicular seminomas was found also to have testicular microlithiasis. The ultrasound and pathological features are described. R o b e r t s , I.S.D. & L o u g h r a n , C . F . (1993). ClinicalRadiology 47, 65-67. Case R e p o r t : T h e U l t r a s o u n d A p p e a r a n c e s o f Testicular Microlithiasis ( ' S n o w S t o r m ' Testis): A Case C o m p l i c a t e d by Testicular Seminoma
A t u l t r a s o u n d the n o r m a l testis shows h o m o g e n e o u s m e d i u m level reflectivity which presents an excellent b a c k g r o u n d for the detection o f testicular t u m o u r s . I n the m a j o r i t y o f such cases, the t u m o u r is localized and usually presents as an a r e a o f either reduced o r m i x e d reflectivity. W e wish to r e p o r t a case o f a p a t i e n t with b i l a t e r a l s e m i n o m a s in which this typical p a t t e r n was superimposed u p o n w i d e s p r e a d highly reflective foci. This p a t t e r n has previously been described in testicular microlithiasis, a rare c o n d i t i o n in which calcified deposits are laid d o w n in a t r o p h i c seminiferous tubules. W e believe this is the first r e p o r t o f the two c o n d i t i o n s occurring in the same patient a n d wish to d r a w a t t e n t i o n to the u n u s u a l u l t r a s o u n d a p p e a r a n c e s which c h a r a c t e r i z e d the case.
CASE REPORT A 26-year-old male presented with a 6 month history of a swollen left testis. There was no past history of cryptorchidism and on examination both testes were intrascrotal. The left testis measured 8 cm in diameter and was hard. An ultrasound study of the scrotum was performed. This confirmed enlargement of the testis which had a diffuse abnormal pattern, consisting of hypoechoic nodules 0.5 1.0 cm in size. The dominant feature, however, was the presence of widespread highly reflectivefoci distributed throughout the testicular parenchyma (Fig. 1). Two larger but similar hypoechoic nodules, measuring approximately
1.5 cm in diameter, were shown in the lower pole of the right testis, but again the dominant feature was the presence of widespread highly reflective foci (Fig. 2). Alpha feto protein levels (< 5 ng/ml on two occasions) were normal as were serial human chorionic gonadotrophin (HCG) estimations (<5 i.u./l). The chest X-ray was normal. He subsequently underwent bilateral orchidectomy.
PATHOLOGY T h e left testis weighed 154 g a n d m e a s u r e d 9 x 6 x 4 cm in size. I t was t o t a l l y r e p l a c e d b y s e m i n o m a (Fig. 3). H i s t o l o g i c a l l y , foci o f calcification were s h o w n t h r o u g h o u t the testis, o c c u r r i n g m a i n l y within the c o l l a g e n o u s t u m o u r stroma. E a c h calcific focus was spherical a n d m e a s u r e d a b o u t 300 /~m in d i a m e t e r . T h e testis was r a d i o g r a p h e d following r e m o v a l a n d this c o n f i r m e d the w i d e s p r e a d n a t u r e o f the calcific foci (Fig. 4). The right testis weighed 53 g a n d m e a s u r e d 5.5 • 3.5 x 3 c m in size. T h e t u m o u r was localized to the lower p o l e a n d m e a s u r e d 3 x 2 x 1 c m in size. Histologically, this was also semin o m a a n d c o n t a i n e d similar calcific foci. Sections o f nont u m o r o u s testis d e m o n s t r a t e d w i d e s p r e a d calcified intrat u b u l a r structures identical in a p p e a r a n c e to those within the tumQur (Fig. 5). The tubules s h o w e d v a r y i n g degrees o f a t r o p h y which was m o s t m a r k e d in those c o n t a i n i n g calcific deposits. A small p r o p o r t i o n o f tubules c o n t a i n e d m a t u r e s p e r m a t o z o a . N o element o f i n t r a t u b u l a r carci-
Fig. 1 - Left testis. Multiple small (2-3 mm) highly reflective foci are shown throughout the testicular parenchyma. Correspondence to: Dr C. F. Loughran, Department of Radiology, Macclesfield District General Hospital, Victoria Road, Macclesfield, Cheshire.
Fig; 2 - Right testis. Again multiple bright foci are shown throughout the testis. Note the presence of tumour nodules in the lower pole of the testis.
66
CLINICAL RADIOLOGY
DISCUSSION
Fig. 3 - Resected specimen (left testis). The cut surface reveals the testis to be almost entirely replaced by seminoma.
Fig. 4 - Radiograph of the excised left testis. Note the widespread calcific deposits throughout the testicular parenchyma.
7t
v
Fig. 5 - Atrophic seminiferous tubules containing calcified deposits.
noma was identified. Both tumours showed multiple small and large areas of necrosis, seen as pale areas in the gross specimen (Fig. 3). No teratomatous elements were noted in either tumour. He is now under regular review and currently remains well with no evidence of tumour recurrence or spread.
Ultrasound of the scrotum is an accurate and safe means of evaluating intrascrotal pathology. Use of transducers with high frequency probes of 7.5-10 MHz enables detailed anatomic depiction of testicular structure and disease. This improves diagnostic a c c u r a c y 25% of tumours are incorrectly diagnosed at the initial physical examination. In assessing testicular tumours ultrasound has proven reliable. It clarifies the nature of a palpable mass and helps determine whether it is intra- or extra-testicular. Reports indicate that in terms of sensitivity and specificity accuracy rates in excess of 95% are possible in the best hands [1]. Tumours of the testis are characteristically welldefined, predominantly hypo-reflective lesions. Generally, it is not possible to distinguish cell types on the basis of the ultrasound appearance, although seminomas usually have a more uniform pattern whereas teratomas tend to be more disorganized with foci o f high reflectivity within them. In analysing the sonographic changes noted in 29 patients with testicular neoplasia Grantham et al. [2] considered three factors of significance when diagnosing tumour by ultrasound: mass, bright echogenic foci and diffuse parenchymal testicular change. Of interest is the observation that bright echogenic foci were visible in 19 (66%) of their patients. Four patients had ultrasoundguided biopsies of these foci; one was due to a focus of cartilage and bone in a malignant teratoma, another due to a calcific focus in a mature teratoma and two in areas of non-calcific scarring in association with regressed germ cell tumours. In the case described, the most prominent characteristic was the widespread echogenic speckling. The underlying pathological cause for the speckling was the deposition o f calcium in the testicular parenchyma. Histological examination demonstrated that the calcifications had developed within atrophic seminiferous tubules and these had subsequently become incorporated within the tumour. Diffuse intratubular calcification as seen here has been termed testicular microlithiasis. This is a rare condition, characterized by the presence throughout the testis of innumerable tiny deposits of calcium within degenerating tubular cells. Cases have been noted in associated with cryptorchidism, Klinefelter's syndrome and male pseudohermaphroditism [3,4]. It has also been described in a patient with pulmonary alveolar microlithiasis and has been noted in normal scrotal testes [5]. Testicular microlithiasis has been previously described at scrotal ultrasound. Characteristically, as in this case, many highly reflective deposits were shown distributed throughout the testicular parenchyma [5,6]. Focal intratubular calcification has been previously recognized histologically around testicular tumours [7]. Kragel et al. [8] recently reported a case of intratubular germ cell neoplasia associated with numerous intratubular microliths but to our knowledge this is the first report of a testicular tumour being associated with the typical sonographic features of testicular microlithiasis. Bilateral testicular seminoma is rare, with a frequency of less than 1% of testicular tumours [9]. Synchronous tumours are even less common, accounting for about one third of bilateral tumours. A further unusual feature of this case is the young age of the patient at presentation, seminomas typically occurring towards the end o f the fourth decade. The aetiology of testicular tumours is not
ULTRASOUND APPEARANCES OF TESTICULAR MICROL1THIASIS
certain but many cases are associated with gonadal dysgenesis as occurs in cryptorchid testes. The presence of bilateral tumours and young age of the patient in this case suggests the possibility of an intrinsic testicular abnormality predisposing to tumour development. The aetiology ofintratubular microliths is also obscure. It has been suggested that they reflect testicular immaturity [10]. In this case it is tempting to speculate that testicular microlithiasis predated the development of tumour and predisposed the patient to it. Whatever the relationship of microlithiasis to tumour, caution should be taken when interpreting such sonographic appearances; an underlying neoplasm should be carefully excluded and follow-up ultrasound screening should be considered. REFERENCES 1 Fowler RC, Chennels PM, Ewing R. Scrotal ultrasonography: a clinical evaluation. British Journal of Radiology 1987;60:649-654.
67
2 Grantham JG, Charboneau JW, James EM, Kirschling RJ, Kvols LK, Segura JW, Wold LE. Testicular neoplasms: 29 tumours studied by high resolution ultrasound. Radiology 1985; 157:775-780. 3 Bieger RC, Passarge E, McAdams AJ. Testicular intratubular bodies. Journal of Clinical Endocrinology 1965;25:1340-1346. 4 Nistal M, Paniagua R, Diez-Pardo JA. Testicular microlithiasis in two children with bilateral cryptorchidism. Journal of Urology 1979;121:535-537. 5 Jaramillo D, Perez-Atayde A, Teele RL. Sonography of testicular microlithiasis. Urologic Radiology 1989; 11:55 57. 6 Doherty F J, Mullins TL, Sant GR, Drinkwater MA, Ucci AA. Testicular microlithiasis. Journal of Ultrasound in Medicine 1987;6:389-392. 7 Azzopardi JG, Mostofi FK, Theiss EA. Lesions of testes observed in certain patients with widespread choriocarcinoma and related tumours. American Journal of Pathology 1961;38:207-219. 8 Kragel PJ, Delvecchio D, Orlando R, Garvin DF. Ultrasonographic findings of testicular microlithiasis associated with intratubular germ cell neoplasia. Urology 1991;37:66-68. 9 Mostofi FK, Price EB. Tumours of the male genital system. Armed Forces Institute of Pathology, 2nd series, 1973. 10 Priebe CJ, Garret R. Testicular calcification in a 4-year old boy. Paediatrics 1970;46:785-788.