Large cell calcifying Sertoli cell tumor (LCCST) is a rare testicular tumor, usually occurring
in young men before the age of 20. Ultrastructural study has been performed
rarely demonstrating Sertoli cell features. We report a case of LCCST in a 7-year-old
boy. A well-circumscribed yellowish-tan, 1.5-cm-sized mass was located within the
left testicular parenchyma. The cut surface was somewhat resilient with multiple calcifications.
On microscopic examination, the tumor was composed of large
eosinophilic polygonal cells with abundant eosinophilic cytoplasm arranging in solid
tubular and trabecular patterns, superficially mimicking Leydig cell tumor. However,
there were multiple calcifications and the tumor cell nests were surrounded by PASpositive
basement membrane. Ultrastructural examination revealed the features of
the Sertoli cell with Spangaro's crystals. The right testis showed a normal appearance
by ultrasonogram and there were no other clinical features associated with Carney
syndrome.ope

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International Journal of Surgical Pathology

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International Journal of Surgical Pathology 7(2):109-114, 1999Large Cell Calcifying Sertoli Cell Tumorof the TestisCase Report with Ultrastructural StudyJeong Hae Kie, MD,* Young Nyun Park, MD, PhD,*Sang Won Han, MD, PhD,t Nam Hoon Cho, MD, PhD,*and Jae Yoon Ro, MD, PhDtLarge cell calcifying Sertoli cell tumor (LCCST) is a rare testicular tumor, usually oc-curring in young men before the age of 20. Ultrastructural study has been performedrarely demonstrating Sertoli cell features. We report a case of LCCST in a 7-year-oldboy. A well-circumscribed yellowish-tan, 1.5-cm-sized mass was located within theleft testicular parenchyma. The cut surface was somewhat resilient with multiple cal-cifications. On microscopic examination, the tumor was composed of largeeosinophilic polygonal cells with abundant eosinophilic cytoplasm arranging in solidtubular and trabecular patterns, superficially mimicking Leydig cell tumor. However,there were multiple calcifications and the tumor cell nests were surrounded by PAS -positive basement membrane. Ultrastructural examination revealed the features ofthe Sertoli cell with Spangaro's crystals. The right testis showed a normal appearanceby ultrasonogram and there were no other clinical features associated with Carneysyndrome. Int J Surg Pathol 7(2):109-114, 1999Key words: large cell calcifying sertoli cell tumor, electron microscopy, Carney syn-drome, calcifications, Leydig cell tumor.Large cell calcifying Sertoli cell tumor (LCCST) ofthe testis is a rare variant of Sertoli cell tumor,which has unique clinical findings [1-6]. This neo-plasm occurs primarily during the first two decadesof life. More than half of the cases show bilateralityand multifocality, and it may be associated withCarney syndrome. The characteristic features of theLCCST are large cells with abundant cytoplasm andcalcifications.From the Departments of *Pathology and tUrology, YonseiUniversity College of Medicine, Seoul, and tDepartment ofPathology, Asan Medical Center, Seoul, Korea.Received for publication July 14, 1998; accepted in revisedform Oct. 30, 1998.Reprint requests: Young Nyun Park, MD, Department ofPathology, Yonsei University College of Medicine, #134, Shin-chon-Dong, Seodaemun-Gu, Seoul, 120-752, Korea.LCCST is frequently misinterpreted as Leydig celltumor because of its histologic resemblance includ-ing large cells with abundant eosinophilic cyto-plasms and prominent nucleoli [7]. The correct di-agnosis must be made on the basis of ultrastructuralfindings as well as the characteristic histologic find-ings including laminated calcifications and in-tratubular growth. We report a case of large cell cal-cifying Sertoli cell tumor in a 7-year-old boy withultrastructural study.Case ReportA 7-year-old boy was admitted because of an in-cidentally found palpable nontender left testicularmass. Ultrasonography revealed a 2.3-cm-sized hy-perechoic mass with multiple calcifications in the109 at PENNSYLVANIA STATE UNIV on September 16, 2016ijs.sagepub.comDownloaded from 1 10 International Journal of Surgical Pathology Vol. 7 No. 2 April 1999left testis while the right testis showed no abnor-mality. Physical examination revealed no other ab-normal findings. Serum alpha-fetoprotein and hu-man chorionic gonadotropin were within normallimits. The patient underwent left orchiectomy un-der the impression of Leydig cell tumor. During theoperation, frozen section examination was per-formed in the biopsied tissue from the mass and thediagnosis of Leydig cell tumor was rendered. Thepostoperative course was uneventful. The patient isalive and well 11 months after operation.Materials and MethodsThe left radical orchiectomized testis was exam-ined grossly and representative tissue sections weretaken for microscopic examination. They were rou-tinely processed and stained with hematoxylin andeosin and periodic acid-Schiff stains. Immunohisto-chemistry was performed on paraffin-embedded tis-sue sections by use of a peroxidase antiperoxidasemethod. Antibodies used for this study included Ki-67 (Dako, Carpinteria, CA) diluted at 1:50, PCNA(Dako) diluted at 1:100, S-100 (Dako) diluted at1:20, vimentin (Dako) diluted at 1:300, AE1/AE3(ZYMED, San Francisco, USA) diluted at 1:50, EMA(Dako) diluted at 1:200, and P53 (Novocastra, New-castle, United Kingdom) diluted at 1:50. Quantifica-tion of Ki-67 and proliferating cell nuclear antigen(PCNA) staining was done by the method of Kratzeret al. [8]. Electron microscopic study was performedon the formalin-fixed tissue, which was postfixed in3% glutaraldehyde with 0.2 mole/L sodium cacody-late buffer at pH 7.4, postfixed with 1% osmiumtetroxide, dehydrated in graded steps of ethanol,cleared with propylene oxide, and embedded inepon resin. A 1-micron section was cut and stainedwith methylene blue and azure II to identify repre-sentative areas for ultrathin sectioning. Ultrathinsections were cut and stained with uranyl acetateand lead citrate and were examined under a Philips300 electron microscope.ResultsGross FindingsThe left testis measured 4.5 x 3 cm. A well-circumscribed, yellowish-tan 1.5-cm-sized masswas located within the parenchyma of the testis. Itssurface was somewhat resilient with multiple calci-fications (Fig. 1). There was no necrosis or hemor-rhage. The epididymis and spermatic cord were un-remarkable.Fig. 1. Cut surface of the left testis shows awell-circumscribed tumor within the testicularparenchyma. The testicular appendage is unre-markable.Light Microscopic FindingsOn histologic examination, the tumor was welldemarcated from the surrounding testicular tissueand was associated with slight lymphocytic infiltra-tion at the periphery of the tumor. The tumor cellsformed solid tubules, trabeculae, cords, and smallnests. The stroma was loose and myxoid or some-what hyalinized. Multiple laminated calcificationswere seen within the tumor nests and stroma (Fig.2). The neoplastic cells were large and round topolygonal with abundant eosinophilic cytoplasms.The nuclei were eccentric with relatively distinct nu-clear membrane, fine chromatins, and prominentnucleoli (Fig. 3). Neither Reinke crystalloids norCharcot-Bottcher crystals were found. The tumorcell nests as well as individual cells were surroundedby PAS-positive, diastase-resistant, basement-mem-brane-like material. Necrosis, mitosis, or vascular in-vasion of the tumor cells was not observed. at PENNSYLVANIA STATE UNIV on September 16, 2016ijs.sagepub.comDownloaded from Large Cell Calcifying Sertoli Cell Tumor 0 Kie et al. 111Fig. 2. Low magnification of LCCST shows tumor cells arranging solid tubules,traberculae, and cords within the myxoid loose stroma. Multiple calcifications areseen (H&E, x40).Fig. 3. The neoplastic cells are large, round to polygonal, with eccentricallylocated nucleoli (H&E, x200).Immunohistochemical FindingsThis case showed diffuse strong reactivity for vi-mentin and focal strong reactivity for S-100. Theimmunohistochemical stains for EMA, p53, andAE1/AE3 were negative. Ki-67 expression valuewas 0.9% and PCNA expression value was 2.9%.Electron Microscopic FindingsElectron microscopic study revealed neoplasticcells forming mostly solid nests, or tubule-like struc-tures with surrounding basal lamina. The tumorcells were connected laterally by easily discerniblejunctions, and in some areas, their plasmalemmas at PENNSYLVANIA STATE UNIV on September 16, 2016ijs.sagepub.comDownloaded from 1 12 International Journal of Surgical Pathology Vol. 7 No. 2 April 1999Fig. 4. (A) Neoplastic cells with irregular nuclei and prominent nucleoli form a solid nest surrounded by basal lamina. Thereare abundant organelles including smooth and rough endoplasmic reticulae, mitochondria, and lipid droplets in the cyto-plasm (uranyl acetate and lead citrate, x7,800). (B) High-power magnification details the surrounding basal lamina and abun-dant cytopasmic organelles (uranyl acetate and lead citrate, x17,800).were slightly interdigitated. The nuclei were oftenirregularly shaped with deep indentations and largenucleoli. The cytoplasm contained abundant or-ganelles including smooth and rough endoplasmicreticula, mitochondria, and lipid droplets (Fig. 4). Afew ribosome-lamella complexes with hollow cylin-drical configuration or parallel pattern were seen. Abundle of intracytoplasmic filaments was found in atumor cell, and it measured 1 ,um long and 0.1 jImwide, which was consistent with Spangaro's crystal(Fig. 5).DiscussionLarge cell calcifying Sertoli cell tumor (LCCST)was first described in 1980 by Proppe and Scully [1].To the present, about 47 cases have been reported[8]. The term large cell calcifying Sertoli cell tumor wasselected for these neoplasms because there weretwo characteristic features, large cells with abun-dant cytoplasm and calcification, which were pre-sent to some extent in all cases.The histogenesis of the LCCST still remains un-certain; however, most reports state that Sertoli cellis the origin of this tumor. One of the major differ-ential diagnoses is Leydig cell tumor, and the cur-rent case was initially misinterpreted as Leydig celltumor on frozen section because of large polygonalcells with relatively abundant eosinophilic cyto-plasm. Later, we diagnosed it as LCCST on perma-nent section on the basis of several light microscopicfeatures. First, multiple calcifications were seen inthe tumor. Second, neoplastic cells formed the solidtubules, trabeculae, and cords, which are generalcharacteristics of LCCST [9]. Third, many clusters oftumor cells as well as individual large cells were sur-rounded by PAS-positive basement membrane[3,6]. Fourth, Reinke crystalloids were not observedin the tumor [9]. Recently, Kratzer et al. reported amalignant form of this tumor, and the criteria in-cluded were a size more than 4 cm, extratesticulargrowth, gross or microscopic necrosis, high-gradecytologic atypia, and vascular invasion [8]. Theyclaimed that the presence of at least two features at PENNSYLVANIA STATE UNIV on September 16, 2016ijs.sagepub.comDownloaded from Large Cell Calcifying Sertoli Cell Tumor 0 Kie et al. 1 13Fig. 5. Electron microscopic finding shows (A) ribosome-lamella complexes with hollow cylindrical configuration and par-allel pattern (uranyl acetate and lead citrate, xl,500) and (B) a bundle of intracytoplasmic filaments (Spangaro's crystal)(uranyl acetate and lead citrate, x3,900).among these criteria indicates malignancy, and thepresence of any one of them, especially in a patientmore than 25 years of age, should be suspicious ofmalignant behavior. This case had none of thesefeatures.The ultrastructural feature of LCCST has beenrarely described [2-4,6,9], and the features of thecurrent case were consistent with Sertoli cell originof this tumor. Most neoplastic cells were arranged insolid, tubule-like structures surrounded by basallamina. The cytoplasm had abundant lipid dropletsand smooth endoplasmic reticulum, which are thecharacteristics of Sertoli cells. Some tumor cellsshowed notched nuclei, which have also been de-scribed as a characteristic finding of normal as well asneoplastic Sertoli cells [10]. The diagnostic feature ofSertoli cells is the presence of Charcot-Bottcher crys-tals (10 to 25 pm long and 2 to 3 pm wide) [11 ]. Thiscase showed aggregated intracytoplasmic filaments,measuring 1 pm long and 0.1 pm wide. It was con-sistent with Spangaro's crystals, which have beensuggested as a precursor form of Charcot-Bottchercrystals [11,12]. The ribosome-lamella complexeswere also found in our case. They have been de-scribed in cases of hairy cell leukemia, and less com-monly in other hematopoietic disorders, as well as insteroid-producing cells and LCCST [3]. Annulatelamellae described in the Sertoli cells in normaltestes were not found in this case.LCCST occurs usually before the age of 20 withbilaterality and multifocality in many cases [5,9].Also, it may be associated with Carney syndrome inwhich patients develop lentigines of the face; myx-omas of the heart, skin, soft tissue, and elsewhere;myxoid fibroadenomas of the breast; blue nevi of theskin; pigmented nodules of the adrenal cortex asso-ciated with Cushing's syndrome; growth-hormone-producing adenomas of the pituitary gland; andpsammomatous melanotic schwannomas. The pre-senting case did not show any other associated clin-icopathologic manifestation. In view of the youngage of the patient, the possibility of late onset of theclinical symptoms cannot be excluded, and there-fore, careful clinical follow-up is warranted. at PENNSYLVANIA STATE UNIV on September 16, 2016ijs.sagepub.comDownloaded from 1 14 International Journal of Surgical Pathology Vol. 7 No. 2 April 1999In conclusion, LCCST is a rare testicular neoplasmwith typical histologic and ultrastuctural features ofSertoli cell origin, although there are some similar-ities of microscopic features with Leydig cell tumor.References1. Proppe KH, Scully RE. Large cell calcifying Sertoli celltumor of the testis. Am J Clin Pathol 74:607-619,19802. Horn J, Jao W, Keh PC. Large cell calcifying Sertolicell tumor of the testis: A case report with ultra-structural study. Ultrastruct Pathol 4:359-364,19833. Perez-Atayde AR, Nunez AE, Carroll WL, MurthyASK, Vaitukaitis JL, Watson DJ, Bauer SB, Koza-kewich HP. Large cell calcifying Sertoli cell tumor ofthe testis. An ultrastructural, immunocytochemi-cal, and biochemical study. Cancer 51:2287-2292,19834. Waxman M, Damjanov I, Khapra A, Landau SJ. Largecell calcifying Sertoli cell tumor of the testis. Lightmicroscopic and ultrastructural study. Cancer54:1574-1581, 19845. Buchino JJ, Uhlenhuth ER. Large-cell calcifying Ser-toli cell tumor. J Urol 141:953-954, 19896. Tetu B, Ro JY, Ayala AG. Large cell calcifying Sertolicell tumor of the testis: A clinicopathologic, im-munohistochemical, and ultrastructural study oftwo cases. Am J Clin Pathol 96:717-722, 19917. Bostwick DG, Eble JN. Urologic surgical pathology,neoplasm of the testis, 1st ed. Mosby-Year Book, St.Louis, 19978. Kratzer SS, Ulbright TM, Talerman A, Srigley JR, RothLM, Wahle GR, Moussa M, Stephens JK, Millos A,Young RH. Large cell calcifying Sertoli cell tumor.Contrasting features of six malignant and six be-nign tumors and a review of the literature. Am JSurg Pathol 21:1271-1280, 19979. Proppe KH, Dickersin GR. Large cell calcifying Sertolicell tumor of the testis: Light microscopic and ul-trastructural study. Hum Pathol 13:1109-1114,198210. Ramzy I, Bos C. Sertoli cell tumor of ovary: Light mi-croscopic and ultrastructural study with histoge-netic considerations. Cancer 38:2447-2451, 197611. Ghardially FN. Ultrastructural pathology of the celland matrix, 4th ed. Butterworth-Heinemann,Stoneham, MA, 199712. Fawcett, DW. Ultrastructure and function of the Ser-toli cell. In Hamilton DW, Greep RO (eds): Hand-book of physiology, vol.5. American PhysiologicalSociety, Baltimore, Maryland, 1975, p. 21 at PENNSYLVANIA STATE UNIV on September 16, 2016ijs.sagepub.comDownloaded from 

Large Cell Calcifying Sertoli Cell Tumor of the Testis : Case Report with Ultrastructural Study

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Large Cell Calcifying Sertoli Cell Tumor of the Testis : Case Report with Ultrastructural Study

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