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2018, Cilt 57, Sayı 3, Sayfa(lar) 178-180
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Primary carcinoid tumor of the testis: A rare case
Gülruh Büberal1, Banu Sarsik2, Adnan Şimşir3, Sadık Tamsel4, Sait Şen2
1İzmir Alsancak Nevvar Salih İşgören State Hospital, Clinic of Pathology, İzmir, Turkey
2Ege University Faculty of Medicine, Department of Medical Pathology, İzmir, Turkey
3Ege University, Department of Urology, İzmir, Turkey
4Ege University Faculty of Medicine, Department of Radiology, İzmir, Turkey
Keywords: Testis, carcinoid tumor, neuroendocrine tumors
Abstract
Neuroendocrine tumors most commonly occur in the gastrointestinal tract, lungs and pancreas. Primary testicular neuroendocrine tumors are rare, constituting 0.23% of all testicular tumors. A 35-year-old man presented to urology department with a painless left scrotal mass. He had no history of trauma. Scrotal Doppler ultrasonography revealed a 2×1.5 cm, well-circumscribed, solid lesion. Tumor marker levels were normal and staging computed tomography demonstrated no evidence of metastasis. The patient subsequently underwent left radical orchiectomy. The orchiectomy material, grossly showed a 1.4x1.2 cm, well-circumscribed, yellow, solid mass. On histopathologic examination, primary testicular carcinoid tumors cannot be distinguished from well-differentiated neuroendocrine carcinoma. The diagnosis of a primary testicular carcinoid tumor was made after ruling out an extratesticular neuroendocrine tumor using clinical and radiological data. This rare case is presented with clinical, morphological and immunohistochemical features.
  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Introduction
    Neuroendocrine tumors (NETs) were first described by Langhans 1 in 1867. These tumors are usually seen in the gastrointestinal tract, lungs, and pancreas. Primary NETs rarely present in the testis, constituting 0.23% of all testicular tumors. Simon et al. 2 reported the first primary testicular carcinoid tumor (TCT) in 1954, more than 60 cases of TCT have been reported, although it remains an uncommon disease 3.

    In this report, we describe a rare case of a primary carcinoid tumor of the testis

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Case Presentation
    A 35-year-old man with no trauma history presented to urology clinic with a painless left scrotal mass. He denied any weight loss, hematuria or systemic symptoms. Physical examination revealed that the scrotal mass was indurated. Serum lactate dehydrogenase (LDH), β-human chorionic hormone (β-HCG), and alpha-fetoprotein (AFP) levels were within the normal ranges. Scrotal Doppler ultrasonography revealed a 2.0 ×1.5 cm, well-circumscribed, solid lesion. Computed tomography demonstrated no evidence of metastasis. The patient subsequently underwent left radical orchiectomy.

    Grossly, the orchiectomy material showed a 1.4 x1.2 cm well-circumscribed, yellow solid mass. The tumor reached the tunica albuginea but did not invade it, nor did the tumor invade the rete testis or epididymis. On histopathologic examination, the tumor displayed features typical of a carcinoid tumor and the adjacent testicular parenchyma showed no germ cell neoplasia in -situ. The tumor cells were arranged in insular and nesting patterns. The cells had abundant granular, eosinophilic cytoplasm. The nuclei were round-to-oval-shaped with a “salt and pepper” chromatin pattern (Figure-1).


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    Figure 1: The nucleus is characterized by “salt and pepper” chromatin pattern without evidence of marked cytologic atypia or mitotic activity (H&E, ×200).

    One mitosis per 10 high-power fields (HPFs) were observed on phospho-histone H3 (pHH3) immuno-histochemical staining. No teratomatous elements were identified. Immunostaining was positive for neuroendocrine markers (chromogranin, synaptophysin, and CD56), supporting the diagnosis of a carcinoid tumor. Immunohistochemistry showed negative staining for placental alkaline phosphatase, D2-40, SALL-4, CD30, OCT-4, AFP, SOX-2, inhibin α, and calretinin. The Ki67 proliferation index was 1%. As the patient had no NETs in other regions, the final diagnosis was a primary testicular carcinoid tumor. Written informed consent was obtained from the patient for publishing the individual medical records.

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Disscussion
  • References
  • Discussion
    In 1907, a German pathologist named Oberndorfer explored the term carcinoid (karzinoide) to distinguish a group of tumors found in the small intestine 1. The first case of a primary TCT was reported by Simon et al. 3 in 1954. The histogenesis of neuroendocrine neoplasms of the testis remains unclear, although several hypotheses have been proposed. Abbosh et al. 4 suggested that TCTs typically occur in the background of a teratoma. They found that isochromosome 12p and 12p over-representations were present in both the carcinoid tumor cells and in the cells of the co-existing mature teratoma. Mai et al. 5 proposed that TCTs arise from Leydig cells, which may be new members of the diffuse neuroendocrine system. In addition, using immunohistochemical methods, Davidoff et al. 6 found neuronal and neuroendocrine cell substances in the cytoplasm of the interstitial Leydig cells of human testes. These findings support the hypothesis that both carcinoid tumor cells and Leydig cells may have the same cell origin in the primary TCT.

    Tumor grade is used as the basis for prognostic classification systems, including those proposed by the European Neuroendocrine Tumor Society (ENETS) and the World Health Organization (WHO) 7,8. In 2010, the revised WHO classification of neuroendocrine neoplasm defined three tumor grades based on either the mitotic rate (mitoses per 10 HPFs) or the Ki67 index (%):

    Grade 1 (NET G1): 2 mitoses/10 HPFs or Ki67 index <2%
    Grade 2 (NET G2): 2-20 mitoses/10 HPFs or Ki67 index 3–20%
    Grade 3 (NET G3): >20 mitoses/10 HPFs or Ki67 index >20%.

    The term carcinoid tumor is used for NET G1.
    In 2016, testis carcinoid tumors were identified as well-differentiated NETs, as part of the WHO classification for tumors of the urinary system and male genitalia. The cases may be pure primary neoplasm as well as with teratomas or epidermoid/dermoid cysts. Most primary TCTs have a benign clinical behavior, regardless of association with teratomas or epidermoid/dermoid cysts. However, lesions with the morphology of an atypical carcinoid can occasionally display metastatic spread. Atypical carcinoid tumors have either coagulative necrosis or increased mitotic activity, with 2-10 mitoses per 10 HPFs. In a series of 29 cases, 1 of the 3 atypical carcinoid tumors had agressive behavior, with a lymph node metastasis and a lung metastasis 9.

    In this case study, the patient’s tumor showed a mitotic rate of 1 mitosis per 10 HPFs by using phospho-histone H3 (pHH3) immunohistochemical stain and a ki67 proliferation index of 1%. There was no necrosis. So that pathological diagnosis was NET G1.

    The most common presentation of a TCT is a painless testicular enlargement, followed by testicular pain and the presence of a hydrocele. Carcinoid syndrome occurs in 10-20% of all carcinoid tumors. No carcinoid syndrome was observed in this case.

    Grossly, the tumors appear to be yellow/tan-colored and solid, with a firm, cut surface due to desmoplasia. The average tumor size is 4.6 cm (ranging from 1.0 cm to 9.5 cm). On histopathologic examination, there are insular, trabecular patterns of monotonous neoplastic cells, with abundant eosinophilic cytoplasm, round-to-oval nuclei, and a distinct nuclear membrane displaying a “salt and pepper” chromatin pattern. The tumor shows positive immunostaining for chromogranin, synaptophysin, and CD56, and negative immunostaining for placental alkaline phosphatase, CD30, β-HCG, AFP, and epithelial membrane antigen. The Ki67 proliferation index is <1% in tumor cells.

    Morphologically, primary TCTs cannot be distinguished from well-differentiated neuroendocrine carcinoma. The diagnosis of a primary TCT was made after ruling out extratesticular NET, using both clinical and radiology data. The treatment course decided was radical orchiectomy. While carcinoid tumors usually have an excellent prognosis, factors such as large tumor size, the presence of carcinoid syndrome, and distant metastases can worsen the prognosis 10.

    The primary carcinoid tumor of the testis is a rare tumor, and this case will contribute to the literature. It should be exclude the metastasis of TCT, because the primary tumor is indistinguishable from the metastatic ones with the morphological and histological characteristics.

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  • Abstract
  • Introduction
  • Case Presentation
  • Discussion
  • References
  • References

    1) Langhans T. Ueber einen drusenpolyp im ileum. Virchows Arch 1867;38:559-60.

    2) Simon HB, McDonald JR, Culp OS. Argentaffin tumor (carcinoid) occurring in a benign cystic teratoma of the testicle. J Urol 1954;72(5):892-4.

    3) Lubana SS, Singh N, Chan HC, Heimann D. Primary neuroendocrine tumor (carcinoid tumor) of the testis: A case report with review of literature. Am J Case Rep 2015;31(16):328-32.

    4) Abbosh PH, Zhang S, Maclennan GT, et al. Germ cell origin of testicular carcinoid tumors. Clin Cancer Res 2008;14(5):1393-6.

    5) Mai K, Park P, Yazdi H, Carlier M. Leydig cell origin of testicular carcinoid tumor: immunohistochemical and electron microscopic evidence. Histopathology 2006;49(5):548-9.

    6) Davidoff MS, Schulze W, Middendorff R, Holstein AF. The Leydig cell of the human testis--a new member of the diffuse neuroendocrine system. Cell Tissue Res 1993;271(3):429-39.

    7) Rindi G, Klöppel G, Alhman H, et al. TNM staging of foregut (neuro)endocrine tumors: A consensus proposal including a grading system. Virchows Arch 2006;449(4):395-401.

    8) Bosman FT CF, Hruban RH, Theise ND. World Health Organization (WHO) Classification of Tumours of the Digestive System. 4th ed. Geneva; WHO Press; 2010:102-107.

    9) Wang WP, Guo C, Berney DM, et al. Primary carcinoid tumors of the testis: A clinicopathologic study of 29 cases. Am J Surg Pathol 2010;34(4):519-24.

    10) Neely D, Gray S: Primary carcinoid tumour of the testis. Ulster Med J 2011;80(2):79-81.

  • Top
  • Abstract
  • Introduction
  • Case Presentation
  • Discussion
  • References
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