Radiotherapeutic management of recurrent choroid plexus carcinoma with spine metastasis and delayed local brain recurrence in young adult male: A case report

Siddharth Malukar; Maitrik Mehta; Suryanarayana Kunikullaya; Ankita Parikh; Akash Pandya; Tasneem Nalawala; Heena Rathod; Dhara Patel; Krishna Prajapati

doi:10.4103/IJNO.IJNO_7_22

CASE REPORT

Year : 2022 | Volume : 5 | Issue : 2 | Page : 41-44

Radiotherapeutic management of recurrent choroid plexus carcinoma with spine metastasis and delayed local brain recurrence in young adult male: A case report

Siddharth Malukar, Maitrik Mehta, Suryanarayana Kunikullaya, Ankita Parikh, Akash Pandya, Tasneem Nalawala, Heena Rathod, Dhara Patel, Krishna Prajapati
Department of Radiation Oncology, The Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India

Date of Submission	06-Aug-2022
Date of Acceptance	12-Mar-2023
Date of Web Publication	08-Apr-2023

Correspondence Address:
Dr. Siddharth Malukar
Department of Radiation Oncology, The Gujarat Cancer and Research Institute, Ahmedabad 380016, Gujarat
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJNO.IJNO_7_22

Abstract

Choroid plexus tumors or “Plexus-chorioideuscarcinomata” are rare malignancies with only a few reported cases. Choroid plexus carcinoma (CPC) is classified under the World Health Organization grade 3. CPC has fewer chances of occurrence in adults than in children. Recurrent CPCs have even more rare occurrences. Herein, we discuss a treated case of CPC in a 26-year-old adult male with spinal metastasis and delayed occurrence of local brain recurrence treated with radiotherapy for symptoms palliation. Clinical features, radiological findings, histopathological examination, and immunohistochemical findings are important in the diagnosis and management of such lesions. It is important to be aware of and know how to deal with such recurrent lesions.

Keywords: Choroid plexus carcinoma, craniospinal irradiation, recurrent

How to cite this article:
Malukar S, Mehta M, Kunikullaya S, Parikh A, Pandya A, Nalawala T, Rathod H, Patel D, Prajapati K. Radiotherapeutic management of recurrent choroid plexus carcinoma with spine metastasis and delayed local brain recurrence in young adult male: A case report. Int J Neurooncol 2022;5:41-4

How to cite this URL:
Malukar S, Mehta M, Kunikullaya S, Parikh A, Pandya A, Nalawala T, Rathod H, Patel D, Prajapati K. Radiotherapeutic management of recurrent choroid plexus carcinoma with spine metastasis and delayed local brain recurrence in young adult male: A case report. Int J Neurooncol [serial online] 2022 [cited 2023 Jun 1];5:41-4. Available from: https://www.Internationaljneurooncology.com/text.asp?2022/5/2/41/373926

Introduction

Choroid plexus tumors or “Plexus-chorioideuscarcinomata” are rare malignancies with only a few reported cases. Choroid plexus carcinoma (CPC) is classified under the World Health Organization (WHO) grade 3 and has a poor prognosis.^[1] The incidence of choroid plexus tumors is ~0.4%–1% and most of the occurrences are seen in less than 2 years of life.^[2]

CPCs account for 15%–20% of choroid plexus tumors and out of which 80% of the CPCs occur in children, adults have less chance of occurrence.^[3] It arises from the epithelium of the choroid plexus of the ventricles.^[4]They are solid tumors and tend to invade brain parenchyma. Recurrent CPCs have even more rare occurrences. Here, we report a case of recurrent CPC in a 26-year-old male.

Case summary

A 26-year-old male presented in the neurosurgery out-patient department with complaints of headache for 2 months. Magnetic resonance imaging (MRI) of brain was showing altered intensity lesion involving left parietal region. He underwent left parieto-occipital craniotomy and excision of the tumor. On histopathological and immunohistochemical examination, it was diagnosed as CPC, WHO grade 3 of lateral ventricular system with MIB EQ ubiquitin protein ligase 1 labeling index of 15%.^[1] Postoperative noncontrast-enhanced computed tomography scan of brain was suggesting postoperative changes.

Patient was referred to radiotherapy department for further management. MRI screening of whole spine was performed, which was suggestive of normal spine screening. Patient received postoperative radiotherapy as craniospinal irradiation (CSI) 36 Gy in 20 fractions followed by local boost 18 Gy in 10 fractions in conventional fractionations by two-dimensional (2D) method.

After regular follow-up and disease-free survival of 5 years, patient developed complaint of bilateral lower limb weakness. MRI of brain with whole spine screening showed 15 × 15 mm intrathecal lesion in spinal canal with a length of 7.4 cm at levels of L5, S1, and S2 vertebras. He underwent L5–S4 laminoplasty with gross total excision of the intradural tumor. On histopathological and immunohistochemical examination, it was diagnosed as recurrent CPC [Table 1] [Figure 1].

Table 1: Various positive and negative Immunohistochemistry (IHC) markers on immunohistochemical examination

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Figure 1: Sections show a highly cellular tumor that shows malignant cells arranged in a solid, cribriform, and papillary pattern. The tumor cells are round to oval with a moderate degree of pleomorphism, hyperchromatic nuclei, and increased mitotic activity

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Postoperative cerebrospinal fluid (CSF) cytology was negative for malignant cells. Patient was given postoperative radiotherapy 45 Gy in 25 fractions in conventional fractionation by 2D method to postoperative spine region from L4 to S4 vertebral body levels.

Patient developed a complaint of urinary incontinence during follow-up after 4 months. MRI spine was showing extensive vertebral metastasis with pathological collapse of D1, D5, D11, D12, and L2 vertebras [Figure 2]. The patient started on chemotherapy with ifosfamide, etoposide, and carboplatin regimen and received six cycles 21 days apart. After six cycles, MRI of brain and whole spine revealed hemorrhagic metastasis in brain and spine metastasis [Figure 3] and [Figure 4].

Figure 2: MRI shows altered signal intensity lesions suggesting area of hyperintensity at the level of D1, D5, D11, D12, L1, S1 and S2 vertebral bodies

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Figure 3: MRI shows two altered signal intensity lesions in bilateral cerebral hemisphere. Largest lesion was 47 x 42 x 37 mm

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Figure 4: MRI shows one altered signal intensity lesions in left cerebral hemisphere

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The patient then presented with complaints of slurred speech and dementia. Patient was given reirradiation to whole-brain 20 Gy in 10 fractions in conventional fractionation by 2D method. Patient had symptomatic relief following radiotherapy. After radiation, patient started chemotherapy with vincristine plus high-dose methotrexate.

Discussion

Choroid plexus tumors arise from the epithelial lining of cerebral ventricles. The function of the choroid plexus is to produce CSF. Choroid plexus tumors consist of choroid plexus papilloma (WHO grade 1), atypical choroid plexus papilloma (WHO grade 2), and CPC (WHO grade 3).^[1]

CPCs histologically show frequent mitoses, nuclear pleomorphism, and blurring of the papillary pattern with poorly structured sheets of the tumor cells and necrotic areas.^[4],[5] These tumors most frequently spread through CSF.^[6]

Hereditary factors are suspected as the cause of CPC. The characteristic mutation seen is tumor suppressor gene P53.^[7] The average MIB EQ ubiquitin protein ligase 1 labeling index of CPCs is 14% and that of normal choroid plexus is 0.02%–0.06%.^[8]

Management of CPCs includes surgery, radiotherapy, and chemotherapy. Surgery is the management of choice for all CPCs both for primary lesion and metastatic deposits. Complete resection leads to a good prognosis. Overall survival is close to 100% and only less than 10% of tumors have shown a tendency for recurrence.^[9] Survival significantly improves when postoperative radiotherapy is given.^[10],[11]

The usual target volume for radiotherapy, which is used traditionally, is CSI followed by local boost.^[12] Adjuvant treatment with radiotherapy and chemotherapy helps to increase disease-free survival but there are rare chances of recurrence.^[3] As these tumors tend to spread via CSF, spinal metastasis occurs frequently even in patients who received adjuvant treatment in form of CSI or systemic therapy.

In our case, patient developed a metastatic lesion in spine after 5 years of primary treatment with a total dose of 54Gy in 30 fractions to whole brain and spine as per institutional protocol. Therefore, after the departmental discussion, reirradiation to spine was delivered up to 45 Gy of radiation dose. Further reirradiation to brain was given only for the palliation of symptoms at a low dose of radiation.

Conclusion

Management of CPCs in adults primarily includes surgery followed by postoperative radiotherapy in form of CSI. Even after treatment, recurrence in the spine can occur. Delayed recurrence is rare and has been reported in very few cases to date as per literature review.^[13] Radiotherapy can be offered for symptom palliation. The combined use of chemotherapy and palliative radiotherapy contributes to an increase in progression-free survival.

In our reported case, 5-year disease-free survival was 100%, and subsequently, patient developed recurrence in spine and brain, which was further treated with the combined use of palliative radiotherapy and chemotherapy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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