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CASE PRESENTATION. RADIATION ONCOLOGY DEPARTMENT, NCI Heba Salah Soliman Assisstant Lecturer. Personal / past / FAMILY HISTORY . 18 year old young lady Resides in Sharkiya Recently married House wife Nullipara Non-smoker - ve DM , HTN
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CASE PRESENTATION RADIATION ONCOLOGY DEPARTMENT, NCI Heba Salah Soliman Assisstant Lecturer
Personal / past / FAMILY HISTORY • 18 year old young lady • Resides in Sharkiya • Recently married • House wife • Nullipara • Non-smoker • -ve DM , HTN • No previous history of Chemotherapy or Radiation • No previous surgeries • No family history of Cancer
presentation • 18 year old young lady presented with post-coital vaginal bleeding • 2 months duration (dating since marriage) • Seen by a Gynecologist , given unknown medication with no improvement of condition • Radiological Investigations were performed …
CT- ABDOMEN AND PELVIS (2/2010) • Rt. Iliac fossa thin walled unilocular cyst with homogenous fluid content, measures 5x4.5cm mostly representing simple ovarian cyst. • Average size of the uterus with regular margins , homogenous attenuation pattern and no focal mass lesion. • Otherwise Normal study
Mri pelvis (2/2010): • Rt. Adenexal wall circumscribed cystic lesion exerting fluid like signals on all pulse sequences, measures ~ 7x5 cm, likely simple Rt. Ovarian cystic lesion. • No Radiological evidence of uterine or cervical mass lesion. • Otherwise Normal study
Patient seen again by Gynecologist PV revealed a uterine cervix friable lesion Punch Biopsy taken… As Pathology was pending… Patient was referred to ncimarch/2010…
Differential diagnosis of cervical mass with or without post-coital bleeding in a young female • Benign Tumor • Cervical pregnancy • Endocervical polyps • Microglandular hyperplasia • Squamous papilloma • Smooth muscle tumors (leiomyomas) • Mesonephric duct remnants • Endometriosis • Papillary adenofibroma • Heterologous tissue (implants of fetal tissue from previous abortion) • Hemangiomas • Malignant Tumor (eg) embryonal RMS
History & physical examination • History (as previously stated) • Physical Examination: • PS 1 • Chest: Normal • Both SCV : Free • Breasts: Free • Abdomen: No organomegaly or palpable masses or lymph nodes • PV: ulcerative friable lesion of the cervix with bleeding on exam • PR: Free
Labs & radiology • CXR: Normal • CT- Abdomen and Pelvis, MRI Pelvis (as previously stated and shown) • LABS: • CBC: WBC=7.22 / RBC=4.18 / HGB=11.8 / PLT=396 (Normal) • LFTs & KFTs: T.bil=0.6 / Alb=4.0 / ALT=20 / AST=21 (Normal) Urea=10 / Creat=0.6 (Normal) • Urine Analysis: Normal
Confirms size and location of a pelvic mass or other findings • Often due to the muscle relaxation induced by anesthesia; EUA may reveal findings that were not appreciated during ambulatory evaluation • Essential for proper pelvic examination and CLINICAL STAGING in Cancer Cervix • Dorsal Lithotomy position • Necessary equipment available: (eg) Good light source. Speculum of appropriate size. Materials to: obtain cervical cytology, biopsy, test for common infections. Swabs for obtaining samples of vaginal discharge. Large cotton swabs to absorb excess vaginal discharge or blood. Water soluble lubricant, disposable gloves, material to drape the patient….etc
Components of the Exam: • Abdomen and Breasts Examined • External Genitalia (skin, labia, perineum, urethra, vestibule, introitus) • Bartholin, para-urethral glands (not palpable when healthy) • PV / PR (under An. Better palpation, examination: vagina, fornices, cervix,rectum) • Speculum Examination: • Inspection of Vagina (lesions/ anomalies/ atrophic mucosa/ discharge) • Inspection of fornices • Inspection of Cervix (discharge/ lesions…etc) • Bimanual Examination (uterus, vagina..) • Recto-Vaginal Examination (uterus, adenexa, cul-de-sac, uterosacral ligament) • DOCUMENTATION!
International Federation for Gynecology and Obstetrics FIGO STAGING FOR CANCER CERVIX Based on clinical examination: (tumor palpation) findings Investigative proceduresincluding: colposcopy, EUA, cystoscopy, and proctoscopy… Does not incorporate information on LN involv.(imp prognostic factor) Despite not altering stage categories; CT, MRI, recently PET or PET/CT, and invasive surgical staging provide important additional information on the extent of the locoregional, LN involvement and distant-disease status.
Tubulo-papillary Adenocarcinoma grade 2 Pathology report (outside nci)
cancer cervix: possible pathology • Squamous cell carcinoma(~80%) : Keratinising /non keratinising /Spindle cell carcinoma • Adenocarcinoma endocervical type • Variants: signet ring, adenoma malignum, villoglandularpapillary adenocarcinoma • Endometrioid adenocarcinoma • Clear cell adenocarcinoma • Serous adenocarcinoma • Mesonephric adenocarcinoma • Intestinal type (signet ring) adenocarcinoma • Other epithelial tumours: Adenosquamous carcinoma / Adenoid cystic carcinoma/ Glassy cell carcinoma/ Adenoid basal Epithelioma • Small cell carcinoma • Undifferentiated carcinoma • Malignant Mixed Mullerian Tumors • Primary Sarcoma (leiomyosarcoma / RMS/ Stromal sarcoma/ Carcinosarcoma)
Cervix Uteri (exocervix and endocervix): Mesonephric carcinoma SLIDE REVISION (NCI)
Pathological DEFFERINTIAL DIAGNOSIS • Mesonephrichyperplasia • Primary endocervical carcinomas of: mucinous, endometrioid, clear cell, and serous differentiation • Malignant Mixed Mulleriantumors • Uterine tumors resembling sex cord tumors • Endometrial endometrioid adenocarcinomas secondarily involving the cervix.
Mesonephric carcinoma….ORIGIN • Malignant neoplasm that arises from mesonephric remnants. • The mesonephric (Wolffian) duct is one of the paired embryogenic tubules along with the Mullerianduct that drain the primitive kidney (mesonephros) to the cloaca. • In both ♀ ♂ the mesonephic duct gives rise to the trigone of the bladder. Mesonephric duct TestosteroneNo Develops into rete testis, Mesonephricduct regresses efferent ducts, epididymes, Remnantsmay persist as seminal vesicles and epoopheron,Skenesglands,Gartner’sduct vasa deferentia, may give rise to cysts and neoplasms • Remnants can be identified mainly in the lateral walls of up to 22% of uterine cervices
Case reports & literature • Very rare with < 50 cases reported in literature • Age at presentation varies: Cases reported in as young as 18 months old, other reports give ranges: 34 to 71, 52 to 55 , 34 to 73 • Most cases present with abnormal vaginal bleeding and have visible cervical masses, although some patients have not had grossly apparent lesions • Many cases in the literature presented as Stage IB, some are higher stage and aggressive • May involve the cervix in a circumferential fashion, be confined to the lateral or posterolateral aspects of the cervix, or extend to the lower uterine segment. The involvement of the cervical wall is usually transmural and includes mucosal involvement
In one report of cases for which clinical follow up was available (27 cases): • 7 patients died of disease within 0.8 – 9 years • 20 patients were alive at latest follow-up (1.5 – 10 years) • 15patients experienced recurrent disease (2-4, 7, 10) • In a report of 8 patients with Mesonephric carcinoma of cervix, Follow-up in 6 cases: • 3alive without evidence of recurrence at postoperative intervals of 2 to 3 years • 1 with recurrent tumor 1 year after hysterectomy; treated CTH , alive, free at 2 yrs. • 1 with intra-abdominal recurrence at 9 yrs, liver metastases at 11 years • 1Death at 8.5 months; probably due to an independent stage IIc ovarian clear-cell carcinoma • These and prior observations in the literature suggest that malignant mesonephric tumors of the cervix may be more indolent than their müllerian counterparts, from which they should be distinguished. • Mesonephriccarcinomas in this site should also be distinguished from florid mesonephric hyperplasia, with which they are usually associated.
Some authors suggest an indolent course with propensity for multiple recurrences, another suggests aggressive clinical course. • Most patients in the literature (particularly 40s and older) underwent TAH, BSO, however a few treated with CCRT • Little is known on optimal therapy or prognosis of this tumor • At present there is no consensus on a standardized ttt protocol for Malignant Mesonephric tumors of the cervix
Back to our case • 18 year old ♀ • Stage IIB Ca Cervix • Mesonephric carcinoma
Radical Concomitant Chemo-Radiation External Beam Radiation to be followed by HDR Intracavitary Brachytherapy TREATMENT DECISION
Patient received Whole Pelvic Irradiation (EBRT): • Dose: 50 Gy / 25 fractions / 5 weeks • Weekly Cisplatin 20 mg/ m2 • Began XRT 3/4/2010 , ended 11/5/2010 • Vaginal beeding persisted during 1st 3 weeks of ttt • Tolerable acute effects (grade1) eg. Mild erythema of skin, diarrhea, abdominal cramps, proctitis, cystitis; managed with appropriate medication • Encompass primary tumor in Cervix, local tum extensions( parametria/ uterosacrallig./ vagina), draining regional lymphatics, areas of LN involv. • Patient received HDR Intracavitary Brachytherapy (Full Application) • 3 applications : 500cGy x 3 ended 1/7/2010 • Iridium-192 • Addition of BTH permits high XRT dose delivery to cervix and para-cervical tissues, improving pelvic disease control and survival • Out-patient procedure
Xrt Late complications: • Hematochezia / Hematuria (Severe) <3-5% • Small bowel obstruction <3-5% • Atypical Vaginal ulceration or Necrosis (mild to moderate) 5-10% • Progresses to Rectovaginal or Vesicovaginal Fistula in ~ 1% • Proctitis , Rectal stricture, Rectal Ulcer 5-10% • Vaginal foreshortening and stenosis (patients who are not sexually active are advised to use vaginal dilator after ttt to minimize vaginal stenosis • XRT to Pelvis ALWAYS LEADS TO INFERTILITY AND MENOPAUSE!
Follow up of patient • CT-Abdomen and Pelvis and MRI Pelvis 2 months post-ttt (9/2010): Free • EUA 2 months after treatment (6/9/2010): CR …No residual • Vaginal Cytology 2 months after ttt (9/2010): -ve for Malignancy • Patient kept under close follow up on a bi-monthly basis 6 months then every 3 months until present • Each visit PV: Free • Routine Vaginal Cytology: Free • Last CT Abdomen and Pelvis (9/2011): Free • Until present date: Patient Alive, Disease Free, Unfortunately has Infertility / Loss of Ovarian Function…Social problems as a result!
Preservation of ovaries Ovaries are highly sensitive to radiation Infertility and ovarian function loss as low as 6-15Gy fractionated XRT • Cryopreservation (investigational) • Embryo • Oocytes • Ovarian Tissue (followed by re-implantation) • Transplantation of Ovarian Tissue • Autologous heterotropic ovarian transplantation • Non-autologous orthoptic ovarian transplantation • Donor oocytes, Gestational Surrogacy • Ovarian Transposition “Oophoropexy”
Ovarian Transposition “Oophoropexy” • Transposing the ovaries out of the radiation field • Inconsistent success rates varying between 16 and 90 % • Transposition can be performed laparoscopically just before the start of radiation therapy (ovaries Should be marked…personal comm Surgeon & Rad Onc) • For pelvic malignancies, lateral ovarian transposition seems superior to medial • Suggested at least 3 cm from the upper border of the radiation field. • Preservation of the ovarian vasculature is also important • Performing the procedure close to the time of irradiation decreases the chance of failure from ovarian migration back into the field of treatment. • Failures are due to various factors: scatter radiation, vascular compromise, radiation dose, age of the patient, ovarian shielding during ttt • Spontaneous pregnancy may be more difficult, and should the patient need IVF, oocyte collection may have to be performed percutaneously unless the ovaries are returned to their original location by a second operation.
Take home messages • Respect patient’s complaint despite Normal Scans • Clinical Examination / EUA gold standards In Gyne • Cancer patients need to be treated in Specialized Institues “Like our beloved NCI” • NCI is the home of “Expert Pathologists” • “What’s common is common” BUT be prepared for what is RARE • Research a rare case , Learn from Case Reports • Sometimes in Medicine you come across “There is no Standard ttt” • CANCER TTT = MULTI-DISCIPLINARY TREATMENT • This young lady ought to have been offered “Oophoropexy” • QOL is not to be overlooked in the Management of patients.
People are often unreasonable, illogical, and self-centered;Forgive them anyway.If you are kind, people may accuse you of selfish, ulterior motives;Be kind anyway.If you are successful, you will win some false friends and some true enemies;Succeed anyway.If you are honest and frank, people may cheat you;Be honest and frank anyway.What you spend years building, someone could destroy overnight;Build anyway.If you find serenity and happiness, they may be jealous;Be happy anyway.The good you do today, people will often forget tomorrow;Do good anyway.Give the world the best you have and it may just never be enough;Give the world the best you have anyway. You see, in the final analysis, it's all between you and God;It was never between you and them anyway. According to the author, Mother Teresa was so moved by these commandments that she hung them on a wall in her orphanage in Calcutta.
