Sandbox:Sahar

|- ! colspan="2" |Management of |- |Stage I ovarian germ cell tumors |

• Unilateral salpingo-oophorectomy with or without lymphangiography or computed tomography (CT)
• Unilateral salpingo-oophorectomy followed by observation
• Unilateral salpingo-oophorectomy with adjuvant radiation therapy or chemotherapy
• Unilateral salpingo-oophorectomy with conservation of uterus and contralateral ovar is indicated in those who plan for future pregnancies.
• Postoperative lymphangiography or CT is indicated for those who have not had the careful surgical and pathological examination of pelvic and para-aortic lymph nodes during surgery.
• Patients with surgically staged stage IA tumors may be observed carefully after surgery without the need for adjuvant treatment.
• Patients with incompletely staged tumor through surgery or those with higher stages may need adjuvant treatment.

|- |Stage II ovarian germ cell tumors |

• Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant radiation therapy or chemotherapy
• Unilateral salpingo-oophorectomy with adjuvant chemotherapy
  • This option is considered for younger patients and those who desire to preserve their fertility for future pregnancies.
  • Radiotherapy has been associated with ovarian failure.
  • Adjuvant chemotherapy with the platinum-based regimen has replaced radiation therapy except in the rare patient in whom chemotherapy is not considered appropriate.

|- |Stage III ovarian germ cell tumors |

• Unilateral salpingo-oophorectomy with adjuvant chemotherapy
• Second-look laparotomy
  • This strategy is not beneficial in patients with completely resected tumors who receive cisplatin-based adjuvant treatment.
  • Second-look surgery may be beneficial in those whose tumor was not completely resected at the initial surgical procedure and who had teratomatous elements in their primary tumor.

|- |Stage IV ovarian germ cell tumors |

• Total abdominal hysterectomy and bilateral salpingo-oophorectomy
• Unilateral salpingo-oophorectomy with adjuvant chemotherapy
  • Chemotherapy is the preferred treatment in the patient who wants to preserve fertility.

|}

Management options of Other Ovarian Tumors

Surgical options for other ovarian tumors
Stage I ovarian germ cell tumors	Unilateral salpingo-oophorectomy with adjuvant chemotherapy Unilateral salpingo-oophorectomy followed by observation Unilateral salpingo-oophorectomy with conservation of uterus and contralateral ovar is indicated in those who plan for future pregnancies. Chemotherapy is usually done postoperatively in those with ovarian germ cell tumors other than pure dysgerminoma and low grade (grade 1) immature teratoma, but it can also preserved for those whose tumors relapse after the surgery.
Stage II ovarian germ cell tumors	Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy, with or without neoadjuvant chemotherapy Unilateral salpingo-oophorectomy with adjuvant chemotherapy, with or without neoadjuvant chemotherapy Second-look laparotomy
Stage III ovarian germ cell tumors	Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy Unilateral salpingo-oophorectomy with adjuvant chemotherapy
Stage IV ovarian germ cell tumors	Total abdominal hysterectomy and bilateral salpingo-oophorectomy with adjuvant chemotherapy with or without neoadjuvant chemotherapy Unilateral salpingo-oophorectomy with adjuvant chemotherapy with or without neoadjuvant chemotherapy

Intraocular classifications of retinoblastoma and their features

	International Intraocular Retinoblastoma Classification (IIRC)	Intraocular Classification of Retinoblastoma (ICRB)
Group A (very low risk)	Small intraretinal tumors away from foveola and optic nerve 3mm or smaller in the greatest dimension, confined to the retina Located further than 3 mm from the foveola and 1.5 mm from the optic disc	Tumors ≤ 3 mm (in basal dimension or thickness)
Group B (low risk)	Tumors confined to the retina Not in the group A Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.	Tumors > 3 mm (in basal dimension or thickness) or Macular location (≤ 3 mm to foveola) Juxtapapillary location (≤ 1.5 mm to disc) Additional subretinal fluid (≤3 mm from margin)
Group C (moderate risk)	Local disease with minimal subretinal or vitreous seeding with following characteristics: Discrete Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina Local fine vitreous seeding may be present close to the discrete tumor Local subretinal seeding less than 3 mm (2 DD) from the tumor	Tumor with: Subretinal seeds ≤ 3 mm from tumor Vitreous seeds ≤ 3 mm from tumor Both subretinal and vitreous seeds ≤ 3 mm from retinoblastoma
Group D (high risk)	Diffuse tumor with significant vitreous or subretinal seeding Maybe massive or diffuse Subretinal fluid present or past without seeding, involving up to total retinal detachment The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses Diffuse subretinal seeding may include subretinal plaques or tumor nodules	Tumor with: Subretinal seeds > 3 mm from tumor Vitreous seeds > 3 mm from tumor Both subretinal and vitreous seeds > 3 mm from retinoblastoma
Group E (very high risk)	Presence of any one or more of the following poor prognosis features Tumor touching the lens Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment Diffuse infiltrating retinoblastoma Neovascular glaucoma Opaque media from hemorrhage Tumor necrosis with aseptic orbital cellulitis Phthisis bulbi	Extensive tumor filling >50% globe or with Neovascular glaucoma Opaque media from hemorrhage in the anterior chamber, vitreous or subretinal space Invasion of the post-laminar optic nerve choroid (>2 mm), sclera, orbit, anterior chamber

Groups	Features
Group A	Small intraretinal tumors away from foveola and optic nerve 3mm or smaller in the greatest dimension, confined to retina Located further than 3 mm from the foveola and 1.5 mm from the optic disc.
Group B	Tumors confined to the retina. Not in the group A Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
Group C	Local disease with minimal subretinal or vitreous seeding with following caracteristics: Discrete Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina Local fine vitreous seeding may be present close to the discrete tumor Local subretinal seeding less than 3 mm (2 DD) from the tumor
Group D	Diffuse tumor with significant vitreous or subretinal seeding May be massive or diffuse Subretinal fluid present or past without seeding, involving up to total retinal detachment The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses Diffuse subretinal seeding may include subretinal plaques or tumor nodules
Group E	Presence of any one or more of the following poor prognosis features Tumor touching the lens Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment Diffuse infiltrating retinoblastoma Neovascular glaucoma Opaque media from hemorrhage Tumor necrosis with aseptic orbital cellulitis Phthisis bulbi

• On microscopic histopathological analysis, carotid body tumor composed of:
  • The chief or paraganglionic cells composing the predominant part of the tumor and contain eosinophilic granular materials and oval or round nuclei.
  • The supporting or sustentacular cells responsible for the chemoreceptor activity of the carotid body
• The characteristic finding of this tumor is:

• Chief cells Arranged in distinctive pattern called cell balls (zellballen)
• Separated by fibrovascular stroma and surrounded by sustentacular cells
• The cytoplasm is pale and diffuse with occasional presence of the eosinophilic granules.
• The nuclei are round to spindle shape.

• The tumor is highly vascular.
• Although there is no well-accepted histologic criteria for the diagnosis of malignant tumors, worrisome histologic features include:
  • Necrosis
  • Extensive vascular or capsular invasion
  • Increased mitotic activity
  • Atypical mitotic figures

Diagnostic algorithm for Infantile onset glyogen storage disease type II

Features on Gross Pathology	Image
Characteristic findings of carotid body tumor, include: Well-circumscribed with psudocapsule The size of the tumor varies greatly and it may be as large as 10 cm The cutting surface is solid with a smooth, rubbery texture||	Contributed by Paweł Kuźniar in wikimedia.commons

Patient with carotid body tumor

History, Physical examination, and evaluation of cnotralateral side

Patients with age < 50 years Patients with multiple paraganglioma Patients with a positive family history

The rest of the patients

SDHD genetic testing

Presence of SDHD mutation

Absence of SDHD mutation

SDHC and SDHB genetic testing

Presence of SDHC/B mutation

Absence of SDHC/B mutation

All the relatives should be evaluated for the presence of paragnaglioma

whole-body 18F-dihydroxyphenylalanine (F-DOPA) positron emission tomography to assess the presence of other paragangliomas

Presence of other paraganglioma

Absence of other paraganglioma

24-hour urine catecholamines and MRI for biochemical screening

surveillance screening every 5 years