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a case of ?primary neuroendocrine tumor of ovary

a case of ?primary neuroendocrine tumor of ovary

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    tumor clinic tumor clinic Presentation Transcript

    • TUMOUR CLINICUNIT I13-09-2011
    • Mrs. R
      53 years old multiparous post menopausal lady with complaints of
      Lower abdominal pain since 7-8months
      Loose stools since 7-8 months
    • h/o lower abdominal pain/ discomfort off and on intermittent, : 7-8 months
      with no aggravating or relieving factors
      with radiation to the back
      h/o passage of loose stools, 7-8 episodes per day : 7-8 months
      Relieved with conservative management
    • H/o perception of vague abdominal mass since the last 2 months
      H/o off and on headache present : 2 months
      H/o decreased appetite and weight loss ( says lost around 10-15 kg in the past six months)
      No h/o any fever/cough/ hemoptysis
      No h/o melaena or hematochezia
      No h/o any altered bladder habits
    • Menstrual h/o: Post-menopausal since 8yrs
      Peri-menopausal transition was smooth
      Previous cycles: 3-4d/28-30d with avg. flow.
      No h/o dysmenorrhoea
      No h/o of any post menopausal bleeding
    • PAST H/o : k/c/o Hypothyroidism since 4-5 yrs
      On ELTROXIN 125µgms OD
      FAMILY H/o: NS
      OBSTETRIC H/o: P2022
      P1 (29yrs) NVD A & H
      P2 (25years) NVD A & H
      P3 MTP for unwanted pregnancy
      P4 MTP for unwanted pregnancy
    • Was being investigated at private clinic
      TTG Negative
      Stool for occult blood : Negative
      Stool for routine microscopy : Normal
      RFT- 23/0.8
      LFT – 14/30/82
      Sr. B12 : 60
      Sr. Ca+2 : 8.5
      PTI 91%
      CA 125 = 132 IU
    • Chest X Ray : mild Cardiomegaly
      USG(7/4/11) :
      Large complex cystic mass in pelvis with minimal ascites
      Ovarian mass13.5 X 8.5 cm with thick internal septae with few internal echoes
      Both ovaries not seperately visualized
      Min. free fluid seen
      Aorta and IVC normal
    • CECT WHOLE ABDOMEN(15/4/11):
      A large solid cystic, soft tissue mass lesion seen in pelvis and lower abdomen measuring 14.4 X 6.4 X 9.5cm.
      The solid component is moderately enhancing and is seen on the left lateral part of the lesion.
      No obvious calcification seen.
      Fat planes with uterus and right lower ureter are lost with right hydroureteronephrosis.
      It is abutting urinary bladder left ureter, sigmoid colon, and few of small bowel loops.
    • There is a 5.8 X 2.9 X 2.8 cm sized nodular soft tissue mass in mesentry in right lower abdomen with spiculated margins.
      Nodular thickening and stranding of omentum also seen
      Liver enlarged with multiple hypo dense lesions seen in the left lobe(at least 3) and in the right lobe( 2 in number) largest measuring 2.7 X 2.0cm in size.
      GB not visualized (post- cholecystectomy status)
      Pancreas spleen and urinary bladder normal
      IVC and Aorta appear normal
    • No e/o any significant periportal/ mesenteric /retroperitoneal/pelvic lymphadenopathy.
      No free fluid in peritoneal cavity
      No pleural effusion.
      No obvious nodules in visualized lower lung
    • On examination
      Afebrile
      Pulse 92/min
      BP - 120/80mm of Hg
      No pallor/edema/cyanosis/clubbing/lymphadenopathy
      Breast & thyroid – normal
      Chest clear
      P/A examination: a solid irregular ill defined mass of around size of 16 weeks gravid uterus felt in the pelvis and lower abdomen with restricted mobility.
    • Per speculum:
      Cervix taken up
      Greenish discharge present : vaginitis
      Pap taken
      Per vaginam:
      Uterus bulky and could not be felt seperately from the mass
      Through the left fornix solid firm irregular mass of 13 X 14cm
      With restricted mobility, ill defined margins and non tender
      Per rectally: same irregular mass was felt
    • Clinical impression
      Clinical impression : 53 years old post menopausal lady with hypothyroidism with malignant adnexal mass (ovarian) with metastases(liver)
    • Blood group: B Positive
      Hb 9.4gm/dl
      TLC – 8800/cu mm
      Platelets – 4.6lacs
      RFTs – 38/1.1
      PTI - 97%
      FBS = 96mg%
      HIV - NR
      HBsAg – NR
      Urine c/s – sterile
      ECG - normal
    • CA 125 – 132 IU/ml
      CA 19.9 – 1.0
      Mammography- normal
      Upper GI Endoscopy : normal
      Colonoscopy : extrinsic compression of sigmoid colon
    • Planned for NACT i/v/o liver metastases
      FNAC (ultrasound guided) of the right adnexal mass was done:
      A-2829/11: malignant tumour- cellular mild pleomorphism,
      s/o possibly malignant epithelial ovarian tumor
      However, possibility of sertoli cell tumour cannot be ruled out.
    • Taken up for staging laparotomy with TAH with BSO
      INTRA-OP:
      Midline vertical incision extended supra - umbilically
      Abdomen opened
      Ascites was present- fluid was taken for peritoneal cytology
      18 X 12cm mass seen arising from the left ovary
      Adhesions present between the sigmoid colon and the tumor mass were separated.
    • Breach in the serosa of sigmoid colon was sutured
      TAH with BSO was done
      Right tube and ovary were normal
      Pelvic peritoneum was indurated
      Omental cake present – infracolic omentectomy done
      Residual disease :
      omental
      Small 1cm deposits over left cardinal ligament
      Peritoneum
      Abdomen closed in layers
    • Parts of urine output was blood tinged.
      Intra op cystoscopy done- b/l ureteric orifice seen urine reflux seen.
      1 unit blood transfusion was given intra-op.
    • Post-operative period
      Monitored satisfactorily
      1 unit blood was given post op
      Intake/output was maintained
      On I.V Ciplox / Metrogyl
      Had a fever spike on day 3 and had wound soakage
      complaints of multiple episodes of loose stools(15-20 times)
      Had developed hypokalemia – k+ correction given
      Stool sent for c/s- no e/o Cl. Difficiles( adv b GE Cx) negative
      Started on inj. Neurobion forte IM OD X 5days(i/v/o dlow Sr. vit B12)
    • PERITONEAL CYTOLOGY(2092/11): No malignant cells seen
      HISTOPATHOLOGY:
      GROSS:
      MICROSCOPICALLY: tumour arranged in the form of solid nests, islands and focally in tubules with fine fibro vascular septa.
      Tumour cell are mildly pleomorphic with fine granular chromatin and moderate amount of granular eosinophilic cytoplasm.
    • DIAGNOSIS
      Left ovary – Neuroendocrine carcinoma
      Right ovary – metastasis present
      Uterus, cervix and B/L fallopian tubes – free of tumour
      Omentum – tumour deposits
      FINAL DIAGNOSIS : STAGE IIIB
    • DISCUSSION
      Neuroendocrine tumors of ovary
    • CARCINOMA OF THE OVARY: STAGINGCLASSIFICATION USING THE FIGO NOMENCLATURE
      I Growth limited to the ovaries
      Ia
      Growth limited to one ovary;
      no ascites present containing malignant cells;
      no tumor on the external surfaces;
      capsule intact
      Ib
      Growth limited to both ovaries; no ascites
      present containing malignant cells;
      no tumor on the external surfaces;
      capsules intact
      Ic*
      Tumor stage Ia or stage Ib but with tumor on the surface of one or both ovaries;
      Or with capsule ruptured;
      Or with ascites present containing malignant cells or with positive peritoneal washings
    • II Growth involving one or both ovaries with pelvic extension
      IIa
      Extension and/or metastases to the uterus and/or tubes
      IIb
      Extension to other pelvic tissues
      IIc*
      Tumor stage IIa or stage IIb but with tumor on the surface of one or both ovaries;
      Or with capsule(s) ruptured;
      Or with ascites present containing malignant cells
      Or with positive peritoneal washings
    • III Tumor involving one or both ovaries with peritoneal implants outside the pelvis and/or positive retroperitoneal or inguinal nodes; superficial liver metastasis equals stage III; tumor is limited to the true pelvis but with histologically verified malignant extension to small bowel or omentum
      IIIa Tumor grossly limited to the true pelvis with negative nodes with histologically confirmed microscopic seeding of abdominal peritoneal surfaces
      IIIb Tumor of one or both ovaries; histologically confirmed implants of abdominal peritoneal surfaces, none exceeding 2 cm in diameter; nodes negative
      IIIc Abdominal implants 2 cm in diameter and/or positive retroperitoneal or inguinal nodes
    • IV
      Growth involving one or both ovaries with distant metastasis;
      if pleural effusion is present, there must be positive cytological test results to allot a case to stage IV;
      parenchymal liver metastasis equals stage IV
    • Carcinoid/ Neuroendocrine tumors
      Classified under Germ cell neoplasms of 0vary which comprise of
      Germ cell tumors
      Dysgerminoma
      Endodermal sinus tumor
      Embryonal carcinoma
      Polyembryoma
      Choriocarcinomas
    • Teratoma
      Immature (solid, cystic, or both)
      Mature
      • Solid
      Cystic
      Mature cystic teratoma (dermoid cyst)
      Mature cystic teratoma (dermoid cyst) with malignant transformation
      Monodermal or highly specialized
      Struma ovarii
      Carcinoid
      Struma ovarii and carcinoid
      Others
      )
    • Mixed forms (tumors composed of types A–F in any
      possible combination)
    • Carcinoid of the ovary
      Primary Carcinoid tumors of ovary account for less than 5% of all Carcinoid tumors.
      And for less than 0.1% of all ovarian neoplasms.
      Are typically localized (89%)
      Nearly all primary ovarian neoplasms are unilateral
    • The tumours are typically classified as germ cell tumors of the ovary and can be divided into four categories:
      Insular
      Trabecular
      Mucinous
      Mixed
    • Primary Carcinoid tumors typically behave in a benign fashion.
      Most ovarian carcinoids contain the insular pattern, are unilateral and early stage.
      It is important to establish that these are not metastatic carcinoids.
      Insular followed by trabecular are the most common subtypes that metastasize to ovary.
    • Given the rarity of the disease, it is also critical to rule out a metastatic GI Primary that could have metastasized to ovary and tend to present as bilateral ovarian metastases.
      The constipation and hirsutism are thought to be due to the release of peptide YY(PYY) by the tumors.
    • The majority of women with primary ovarian Carcinoid tumours are found incidentally on cross sectional or ultrasound imaging.
      Rarely, they may also present with abdominal pain, constipation, hirsutism, and a pelvic
    • For women who present with a stage I primary ovarian carcinoid, the prognosis is excellent, with greater than 90% survival.
      Women with more advanced diseased, the prognosis poor. (Octreotide has been used in some reports)
    • INSULAR CARCINOID:
      Most common type of ovarian carcinoid.
      Occurring in patients with between 40 & 80 years of age.
      Majority present with a pelvic mass and approximately 40% will have clinical evidence of carcinoid syndrome.
      Those presenting with carcinoid syndrome have larger masses(>7cm).
      It is important to differentiate a primary lesion from a ovarian metastases.
    • Typically slow growing.
      For those limited to the ovary, the 10 yr survival 100%(if unilateral)
      With advanced stage disease, the 5-year survival is 33%.
    • TRABECULAR CARCINOID:
      Second most common
      In women 30 – 70 years of age.
      Not typically associated with carcinoid syndrome, but may be associated with constipation due to PYY.
      No reported tumor related deaths
    • MUCINOUS CARCINOID:
      Rare
      Occur in patients between 30 and 80years of age
      Tumors may be pure or may be associated with mature cystic teratomas.
      One need to consider that the ovarian tumor may be a metastasis from gastrointestinal tumor.
      In mucinous carcinoid, an appendicectomy should be performed to exclude the more common primary mucinous appendiceal carcinoid.
      Overall survival good
    • Management
      The most recent Neuroendocrine tumor NCCN guidelines recommend that the tumor be removed completely with the goal of attaining negative margins.
      If the diagnosis is known or suspected pre-operatively, Octreotide (100-500mcgSQ/IV every 6 – 12h) be administered immediately prior to or during resection to avoid carcinoid crisis.
      In ovarian carcinoid tumors, fertility sparing surgery is permissible as these tumors are generally unilateral and associated with a good prognosis.
      But can include radical debulking surgery dependent on patients age and disease distribution.
    • Most common metastatic sites include regional lymph node, liver, bone and lung.
      Most demonstrate a remarkable tropism for the liver.
      Liver lesions should be considered for resection to control tumor burden and
      NCCN guidelines recommend that those lesions that are not resectable should be considered for cryotherapy, radiofrequency ablation, or regional embolisation.
    • Role of adjuvant therapy
      No evidence to support the use of either hormonal, chemotherapy or radiation therapy for gynecological carcinoid tumours.
    • Advanced stage or Recurrent disease