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| Year : 2016 | Volume
: 59
| Issue : 2 | Page : 249-250 |
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| Choroid plexus carcinoma with hyaline globules: An unusual histological feature |
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Asha Sharad Shenoy, Heena Maulek Desai
Department of Pathology, T. N. Medical College and B. Y. L. Nair Charitable Hospital, Mumbai, Maharashtra, India
Click here for correspondence address and email
| Date of Web Publication | 9-May-2016 |
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How to cite this article:
Shenoy AS, Desai HM. Choroid plexus carcinoma with hyaline globules: An unusual histological feature. Indian J Pathol Microbiol 2016;59:249-50 |
Editor,
Choroid plexus tumors are uncommon intracranial tumors with choroid plexus carcinoma (CPC) constituting 15–20% of them.[1] CPC are diagnosed by frank signs of malignancy, frequent mitosis, nuclear pleomorphism, increased cellularity, necrosis and blurring of the papillary pattern. In addition, CPC can also show unusual histological features such as mucinous degeneration, melanization, amyloid deposition, psammoma bodies, tubular glandular structures and rarely hyaline globules.[2] We report here a case of CPC with numerous hyaline globules.
A 5-year-old boy complained of headache and vomiting since 3 weeks. Magnetic resonance imaging (MRI) showed a 5.6 cm × 3.8 cm × 4.3 cm space occupying lesion occupying a dilated temporo-parietal horn of lateral ventricle. The mass enhanced on contrast and perilesional edema was seen. On histology (H and E), the tumor was arranged in sheets and occasional papillae. The cells were bizarre, many were epithelioid, had abundant eosinophilic cytoplasm with round to ovoid vesicular to hyperchromatic nuclei with prominent nucleoli [Figure 1]. Mitosis was more than 5/10 hpf and necrosis was focal. | Figure 1: Section showing tumor cells arranged in sheets and occasional papillae; many of the cells are epithelioid with abundant eosinophilic cytoplasm. Inset: Numerous bright pink rounded hyaline globules seen (H and E, ×100)
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In addition, there were numerous bright pink rounded hyaline globules seen intra and extracellularly which were Periodic-acid Schiff (PAS) stain positive and resistant to diastase [Figure 2]. The differential diagnosis on H and E stains was CPC, ependymoma, atypical teratoid/rhabdoid tumor (ATRT), yolk sac tumor (YST) and a rare possibility of meningioma. There was no evidence of rosettes or Schiller–Duval bodies. | Figure 2: (a) Periodic acid Schiff positive diastase resistant tumor cells; (b-d) tumor cells showing positivity for vimentin, cytokeratin and INI-1 stains respectively
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On immunohistochemistry (IHC), vimentin (focal) and cytokeratin (CK) were expressed. There was nuclear expression of INI-1 [Figure 2]. Epithelial membrane antigen (EMA), glial fibrillary acid protein (GFAP), synaptophysin, CD-30, alpha-fetoprotein and glypican 3 stains were negative. Mib-1 labeling index was 12–14%. In view of the above histological and IHC markers, the final histopathological diagnosis was given as CPC with numerous hyaline globules (WHO grade III) ruling out YST.
CPCs are rare tumours of childhood and very rarely seen in adults. CPC have been known to have varied unusual histological features. However, hyaline globules is a very rare occurrence seen with CPC.[3] These globules are PAS positive and diastase resistant α-1 antitrypsin and have no prognostic significance. Some studies have observed that laminated hyaline globules can occur in CPC and may be the precursor of the formation of psammoma bodies seen more frequently in CPC.[2] Hyaline globules have also been reported in intracranial malignant peripheral nerve sheath tumour and is proposed to be caused by the abundant vascular network and degeneration of tumor cells.[4]
CPC shares few clinical, histological and immunophenotypic features with other brain tumors. One of them is ATRT which is a very close differential of CPC. ATRT can show heterologous elements such as mesenchymal and epithelial differentiation, whereas CPC shows a more homogenous histological picture. INI-1 expression has been shown to differentiate between the two since its expression is lost in ATRT due to inactivation of the INI-1 gene.[5] In our case, we had diffuse INI-1 positivity favouring a diagnosis of CPC.
CPC has a 5 year survival rate less than 50%. This case highlights the fact that CPC can present with numerous hyaline globules and its possibility should be considered in a patient presenting with an intraventricular mass.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Mishra A, Srivastava C, Singh SK, Chandra A, Ojha BK. Choroid plexus carcinoma: Case report and review of literature. J Pediatr Neurosci 2012;7:71-3.  [ PUBMED]  |
| 2. | Jaiswal S, Vij M, Mehrotra A, Kumar B, Nair A, Jaiswal AK, et al. Choroid plexus tumors: A clinico-pathological and neuro-radiological study of 23 cases. Asian J Neurosurg 2013;8:29-35. [ PUBMED] |
| 3. | Burger PC, Scheithauer BW, Vogel FS, editors. The brain: Tumors. In: Surgical Pathology of the Nervous System and Its Coverings. 4 th ed. New York: Churchill Livingstone; 2002. p. 258-64. |
| 4. | Kim YE, Suh YL, Lee SE, Shin HJ. Intracranial MPNST with multivacuolated cells and hyaline globules: A case with ultrastructural findings. Ultrastruct Pathol 2012;36:195-200. |
| 5. | Judkins AR, Burger PC, Hamilton RL, Kleinschmidt-DeMasters B, Perry A, Pomeroy SL, et al. INI-1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma. J Neurol 2005;64:391-7. |
Correspondence Address:
Heena Maulek Desai
Department of Pathology, T. N. Medical College and B. Y. L. Nair Charitable Hospital, Dr. A. L. Nair Road, Mumbai Central, Mumbai - 400 008, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.182042
[Figure 1], [Figure 2] |
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Effect of Surgery, Adjuvant Therapy, and Other Prognostic Factors on Choroid Plexus Carcinoma: A Systematic Review and Individual Patient Data Analysis |
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