Pineal embryonal carcinoma: Difference between revisions
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==Treatment== | ==Treatment== | ||
The mainstay of therapy for pineal embryonal carcinoma is [[radiotherapy]] and/or [[chemotherapy]]. Sometimes, [[surgical resection]] may be done. | |||
{| class="wikitable" | {| class="wikitable" | ||
|+ | |+ | ||
! colspan="2" |Management Options of Penial Gland tumors | ! colspan="2" |Management Options of Penial Gland tumors | ||
|- | |- | ||
!'''CSF diversion''' | |||
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* The optimal surgical strategy to treat acute [[hydrocephalus]] in patients with pineal tumors is uncertain. | * The optimal surgical strategy to treat acute [[hydrocephalus]] in patients with pineal tumors is uncertain. | ||
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* When CSF diversion is necessary, endoscopic third [[ventriculostomy]] can be carried out at the same time as the biopsy and is preferred over [[Ventriculoperitoneal shunt|VP shunts]], which can be complicated by infection, shunt malfunction, [[subdural hematoma]], and rarely, tumor seeding | * When CSF diversion is necessary, endoscopic third [[ventriculostomy]] can be carried out at the same time as the biopsy and is preferred over [[Ventriculoperitoneal shunt|VP shunts]], which can be complicated by infection, shunt malfunction, [[subdural hematoma]], and rarely, tumor seeding | ||
|- | |- | ||
!'''Surgical resection''' | |||
| | | | ||
* Some series report long-term survival with surgery alone, even in patients with [[Pineoblastoma|pineoblastomas]]. | * Some series report long-term survival with surgery alone, even in patients with [[Pineoblastoma|pineoblastomas]]. | ||
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* Patients with symptomatic recurrent [[pineocytomas]] should also be considered for surgical resection of the lesion. | * Patients with symptomatic recurrent [[pineocytomas]] should also be considered for surgical resection of the lesion. | ||
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!'''Radiation''' | |||
| | | | ||
* Postoperative adjuvant [[radiation therapy]] is frequently (but not universally) recommended, and local control is dose-dependent. | * Postoperative adjuvant [[radiation therapy]] is frequently (but not universally) recommended, and local control is dose-dependent. | ||
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* Adjuvant RT is not universally recommended after gross total resection of a [[pineocytoma]]. | * Adjuvant RT is not universally recommended after gross total resection of a [[pineocytoma]]. | ||
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!'''Stereotactic radiosurgery''' | |||
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*[[Stereotactic radiosurgery|Stereotactic radiosurgery (SRS)]] is emerging as a useful treatment alternative for [[pineocytomas]], although experience is limited. | *[[Stereotactic radiosurgery|Stereotactic radiosurgery (SRS)]] is emerging as a useful treatment alternative for [[pineocytomas]], although experience is limited. | ||
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* Due to the low rate of side effects, IRS may develop into an attractive alternative to microsurgery in de novo diagnosed [[Pineocytoma|pineocytomas]]. In malignant PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional irradiation. | * Due to the low rate of side effects, IRS may develop into an attractive alternative to microsurgery in de novo diagnosed [[Pineocytoma|pineocytomas]]. In malignant PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional irradiation. | ||
|- | |- | ||
!'''Chemotherapy as part of multimodality therapy''' | |||
| | | | ||
* The similarity of [[Pineoblastoma|pineoblastomas]] to [[Medulloblastoma|medulloblastomas]] in terms of their clinical behavior and tendency for leptomeningeal seeding has led to the use of similar chemotherapy regimens in patients with [[pineoblastoma]] as part of a multimodality approach. | * The similarity of [[Pineoblastoma|pineoblastomas]] to [[Medulloblastoma|medulloblastomas]] in terms of their clinical behavior and tendency for leptomeningeal seeding has led to the use of similar chemotherapy regimens in patients with [[pineoblastoma]] as part of a multimodality approach. | ||
Latest revision as of 22:21, 6 November 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2] Sujit Routray, M.D. [3]
Synonyms and keywords: Pineal embryonal cell carcinoma; Pineal gland tumor; Brain tumor
Overview
Pineal embryonal carcinoma is a relatively rare malignant neoplasm and accounts for a small proportion of all intracranial germ cell tumors. It is an aggressive tumor and has a propensity to metastasize systemically. A component of embryonal carcinoma is often found in mixed germ-cell tumors, in which case it is usually the most aggressive component, and dictates prognosis. Pure pineal embryonal carcinoma tumors do not secrete β-HCG or AFP. On microscopic histopathological analysis, pineal embryonal carcinoma is characterized by: Poorly differentiated, pleomorphic cells in cords, sheets, or papillary formation, indistinct cell borders, nucleoli, vesicular nuclei (clear, empty appearing nuclei) and necrosis. If left untreated, patients with pineal embryonal carcinoma may progress to develop seizures, obstructive hydrocephalus, and CSF metastasis. Common complications of pineal embryonal carcinoma include: Obstructive hydrocephalus, leptomeningeal spread, and systemic metastasis. The clinical presentation of pineal embryonal carcinoma is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct. Symptoms of pineal embryonal carcinoma include headaches, nausea, vomiting, seizures, hearing loss, sleepiness, and double vision. Compression of the superior colliculi can lead to a characteristic gaze palsy, known as Parinaud syndrome. Head CT and brain MRI may be diagnostic of pineal embryonal carcinoma. Biopsy is generally done to confirm the diagnosis of pineal embryonal carcinoma. The mainstay of therapy for pineal embryonal carcinoma is radiotherapy and/or chemotherapy. Sometimes, surgical resection may be done. CSF diversion, radiation, and stereotypical surgery are alternative treatment options based on the patient situation.
Pathophysiology
- Pineal embryonal carcinoma is a relatively rare malignant neoplasm and accounts for a small proportion of all intracranial germ cell tumors.
- It is an aggressive tumor and has a propensity to metastasize systemically.
- A component of embryonal carcinoma is often found in mixed germ-cell tumors, in which case it is usually the most aggressive component, and dictates prognosis.
- Pure pineal embryonal carcinoma tumors do not secrete β-HCG or AFP.[1]
- On microscopic histopathological analysis, pineal embryonal carcinoma is characterized by:[2]
- Poorly differentiated, pleomorphic cells in cords, sheets, or papillary formation
- Indistinct cell borders
- Nucleoli - key feature
- Vesicular nuclei (clear, empty appearing nuclei) - key feature
- Necrosis - common
- Mitoses - common
- Variable architecture:
- Solid (predominant in ~55% of cases)
- Glandular (predominant in ~17% of cases)
- Papillary (predominant in ~11% of cases)
- Nested
- Micropapillary
- Anastomosing glandular
- Sieve-like glandular
- Pseudopapillary
- Blastocyst-like
- Embryoid bodies - ball of cells in surrounded by empty space on three sides
- Pineal embryonal carcinoma is demonstrated by positivity to tumor markers such as:[3]
Differentiating Pineal Embryonal Carcinoma From Other Conditions
- Pineal embryonal carcinoma must be differentiated from:
- Pineocytoma
- Pineal parenchymal tumor with intermediate differentiation
- Papillary tumor of the pineal region
- Pineoblastoma
- Pineal germinoma
- Pineal choriocarcinoma
- Pineal yolk sac carcinoma (endodermal sinus tumor)
- Pineal teratoma
- Pineal cyst
- Astrocytoma of the pineal gland
- Meningioma near pineal gland
- Pineal metastasis
- Cavernoma in pineal region
- Aneurysm in pineal region If left untreated, patients with pineal embryonal carcinoma may progress to develop seizures, obstructive hydrocephalus, and CSF metastasis.
Natural History, Complications, and Prognosis
- Pineal embryonal carcinoma is a relatively rare malignant neoplasm and accounts for a small proportion of all intracranial germ cell tumors.
- It is an aggressive tumor and has a propensity to metastasize systemically.
- Common complications of pineal embryonal carcinoma include:
- Obstructive hydrocephalus
- Leptomeningeal spread
- Systemic metastasis
Clinical Findings
- The clinical presentation of pineal embryonal carcinoma is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct.
- Symptoms of pineal embryonal carcinoma include:
- Headaches
- Nausea and vomiting
- Seizures
- Hearing loss
- Sleepiness and confusion
- Double vision
- Compression of the superior colliculi can lead to a characteristic gaze palsy, known as Parinaud syndrome.
- Head CT and brain MRI may be diagnostic of pineal embryonal carcinoma.
- Biopsy is generally done to confirm the diagnosis of pineal embryonal carcinoma.
Treatment
The mainstay of therapy for pineal embryonal carcinoma is radiotherapy and/or chemotherapy. Sometimes, surgical resection may be done.
| Management Options of Penial Gland tumors | |
|---|---|
| CSF diversion |
|
| Surgical resection |
|
| Radiation |
|
| Stereotactic radiosurgery |
|
| Chemotherapy as part of multimodality therapy |
|
References
- ↑ Intracranial embryonal carcinoma. Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-embryonal-carcinoma. Accessed on December 4, 2015
- ↑ Microscopic features of embryonal carcinoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Embryonal_carcinoma. Accessed on December 4, 2015
- ↑ IHC features of embryonal carcinoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Embryonal_carcinoma. Accessed on December 4, 2015