Pineal embryonal carcinoma

Revision as of 22:21, 6 November 2019 by Mmir (talk | contribs) (→‎Treatment)
Jump to navigation Jump to search

WikiDoc Resources for Pineal embryonal carcinoma

Articles

Most recent articles on Pineal embryonal carcinoma

Most cited articles on Pineal embryonal carcinoma

Review articles on Pineal embryonal carcinoma

Articles on Pineal embryonal carcinoma in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Pineal embryonal carcinoma

Images of Pineal embryonal carcinoma

Photos of Pineal embryonal carcinoma

Podcasts & MP3s on Pineal embryonal carcinoma

Videos on Pineal embryonal carcinoma

Evidence Based Medicine

Cochrane Collaboration on Pineal embryonal carcinoma

Bandolier on Pineal embryonal carcinoma

TRIP on Pineal embryonal carcinoma

Clinical Trials

Ongoing Trials on Pineal embryonal carcinoma at Clinical Trials.gov

Trial results on Pineal embryonal carcinoma

Clinical Trials on Pineal embryonal carcinoma at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Pineal embryonal carcinoma

NICE Guidance on Pineal embryonal carcinoma

NHS PRODIGY Guidance

FDA on Pineal embryonal carcinoma

CDC on Pineal embryonal carcinoma

Books

Books on Pineal embryonal carcinoma

News

Pineal embryonal carcinoma in the news

Be alerted to news on Pineal embryonal carcinoma

News trends on Pineal embryonal carcinoma

Commentary

Blogs on Pineal embryonal carcinoma

Definitions

Definitions of Pineal embryonal carcinoma

Patient Resources / Community

Patient resources on Pineal embryonal carcinoma

Discussion groups on Pineal embryonal carcinoma

Patient Handouts on Pineal embryonal carcinoma

Directions to Hospitals Treating Pineal embryonal carcinoma

Risk calculators and risk factors for Pineal embryonal carcinoma

Healthcare Provider Resources

Symptoms of Pineal embryonal carcinoma

Causes & Risk Factors for Pineal embryonal carcinoma

Diagnostic studies for Pineal embryonal carcinoma

Treatment of Pineal embryonal carcinoma

Continuing Medical Education (CME)

CME Programs on Pineal embryonal carcinoma

International

Pineal embryonal carcinoma en Espanol

Pineal embryonal carcinoma en Francais

Business

Pineal embryonal carcinoma in the Marketplace

Patents on Pineal embryonal carcinoma

Experimental / Informatics

List of terms related to Pineal embryonal carcinoma

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:

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:

Clinical Findings


Treatment

Management Options of Penial Gland tumors
CSF diversion
  • The optimal surgical strategy to treat acute hydrocephalus in patients with pineal tumors is uncertain.
  • When CSF diversion is necessary, endoscopic third ventriculostomy can be carried out at the same time as the biopsy and is preferred over 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 pineoblastomas.
  • Indeed, for pineoblastomas, gross total surgical resection appears to correlate with improved survival.
  • Patients with symptomatic recurrent pineocytomas should also be considered for surgical resection of the lesion.
Radiation
  • Postoperative adjuvant radiation therapy is frequently (but not universally) recommended, and local control is dose-dependent.
  • The incidence of leptomeningeal recurrence was significantly lower among patients receiving CSI compared with those who did not.
  • The five-year survival rates were 86 and 49 percent for pineocytomas and non-pineocytoma PPTs, respectively.
  • Adjuvant RT is not universally recommended after gross total resection of a pineocytoma.
Stereotactic radiosurgery
  • Stereotactic radiosurgery (SRS) is emerging as a useful treatment alternative for pineocytomas, although experience is limited.
  • The precise radiation fields that are defined by MRI or CT-computerized treatment planning minimize damage to the surrounding brain, and the risks of general anesthesia and craniotomy are avoided.
  • Stereotactic radiosurgery (SRS) is increasingly being used to treat pineal region tumors, either as an additional therapy after conventional treatments or as a primary treatment.
  • Due to the low rate of side effects, IRS may develop into an attractive alternative to microsurgery in de novo diagnosed 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 pineoblastomas to 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.
  • Chemotherapy has been used to delay radiation therapy in very young children, for whom the long-term neurocognitive and developmental side effects of craniospinal irradiation (CSI) are a major concern.
  • The importance of radiation therapy as a component of the initial treatment of supratentorial primitive neuroectodermal tumors (PNETs) is also supported by the German HIT-SKK87 and HIT-SKK92 protocols, as well as the Canadian pediatric brain tumor protocol.

References

  1. Intracranial embryonal carcinoma. Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-embryonal-carcinoma. Accessed on December 4, 2015
  2. Microscopic features of embryonal carcinoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Embryonal_carcinoma. Accessed on December 4, 2015
  3. IHC features of embryonal carcinoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Embryonal_carcinoma. Accessed on December 4, 2015


Template:WikiDoc Sources