Case 06- Dr. Nisreen Abu Shahin Associate Professor of Pathology - - PowerPoint PPT Presentation

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Case 06- Dr. Nisreen Abu Shahin Associate Professor of Pathology - - PowerPoint PPT Presentation

Feb 11, 2024 286 likes •896 views

Case 06- Dr. Nisreen Abu Shahin Associate Professor of Pathology Department of Pathology, University of Jordan Amman, Jordan Case 24 yr old nulliparus woman, no known medical disorders, not using any medications or hormones presents

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Case 06-

  • Dr. Nisreen Abu Shahin

Associate Professor of Pathology Department of Pathology, University of Jordan Amman, Jordan

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Case

  • 24 yr old nulliparus woman, no known medical

disorders, not using any medications or hormones

  • presents to a general physician clinic with

severe menorrhagia of 2 years duration causing excessive vaginal bleeding and severe anemia

  • She was referred to GYN clinic for evaluation
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Physical examination:

  • Pallor
  • Normal BMI
  • No palpated abdominal masses
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Imaging

  • Pelvic Ultrasound. globular large uterus with

multiple uterine polyps extending into cervical

  • s, with abnormal echogenecity.
  • CT Scan. marked heterogeneous uterine

enlargment suspicoius for adenomyosis, along with a large heterogeneous uterine polyploid mass extending to cervical os

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D&C

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Polypoid mass

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Hyalinization

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Differential diagnosis

  • Uterine tumors resembling ovarian sex cord

tumors (UTROSCT)

  • Malignant mullerian mixed tumors (MMMT)
  • Endometrial stromal tumors
  • Epithelioid smooth muscle tumors
  • Endometrioid carcinoma with weird

morphology

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IHCs

EMA Vimentin

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a-Inhibin

Inhibin

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Calretinin

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PAX 8

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CK7

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ER

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CK5/6 p63

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CD10 Desmin

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SMA

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Diagnosis??

  • Designation from previous reports*:

"corded and hyalinized endometrioid carcinoma" reflecting most striking and consistent features.

  • A phenotype of endometrioid carcinomas

appreciated in recent decades

* Young RH. Am J Surg Pathol. 2005 Feb;29(2):157-66

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Uterus

Hysterectomy was performed

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Complex architecture

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Squamous morules

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Atypia

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ACH

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Adenomyosis

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  • Cervix: unremarkable
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Differential diagnosis???

  • Familial / inherited cancer syndromes ?
  • Ovarian tumors secreting estrogens??
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  • Back to patient’s family history:
  • Parents: consanguineous marriage
  • Mother died in early 40s with cancer

(?multiple; ?? GIT)

  • A brother with GIT polyps
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Ovaries

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  • Frozen sections were performed on right and

left ovaries (Normal sized)

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Right ovary

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Left ovary

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EMA Inhibin

IHCs

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  • Ovarian stroma had multiple nodules showing

annular tubules with central eosinophilic hyaline bodies and peripheral oriented nuclei (greatest diameter is at least 6mm of the examined frozen tissue).

  • Foci of calcification are identified.
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(SEX CORD TUMOR WITH ANNULAR TUBULES)

Bilateral SCTAT

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Comments

  • “The presence of bilateral ovarian sex cord

tumor with annular tubules is a likely source of hyper-estrogenism leading to the pathologies seen in the uterus. Moreover, the presence of the bilateral ovarian tumor raises the possibility of Peutz-Jeghers syndrome. Clinical evaluation for Peutz-Jeghers syndrome is recommended”.

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  • Hyperpigmented lesions over the perioral

region

From literature

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Peutz-Jeghers syndrome (PJS)

  • inherited autosomal dominant cancer syndrome
  • tumor suppressor gene STK11/LKB1, 19p13.3
  • mucocutaneous melanin pigmentation &

hamartomatous GIT polyps

  • Incidence : 1 /50,000 to 1/ 200,000 live births
  • predisposition to: benign and malignant tumors
  • f stomach, small intestine, pancreas, cervix,

breast and ovaries.

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The Diagnostic Criteria for PJS is Clinical

  • Include:
  • small bowel hamartomatous polyps
  • characteristic mucocutaneous pigmentation
  • family history
  • Two of these criteria must be met in order to

make a clinical diagnosis of PJS

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Cancer in PJS

Banno Ee al: GYNECOLOGICAL CANCER IN PJS. Oncology Letters (2013) 6: 1184-1188.

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PJS – GIT tumors

  • Non malignant: P-J hyperplastic polyps; ?

Tubular adenomas

  • malignant GIT:
  • large intestine>> stomach> small intestine >

pancreas (mucinous adenocarcinomas)

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PJS- GYN tumors

  • highest in cervix >> ovary
  • ovarian, cervical and breast cancers: surveillance is

necessary

  • Minimal deviation adenocarcinoma (MDA)
  • SCTAT (“in PJS is commonly multifocal, bilateral, small

(detected microscopically), and calcified in >50% of cases)”* Scully RE et al. Cancer .1982

  • Mucinous and serous epithelial ovarian tumors ↑

(benign, borderline, malignant )

  • lifetime risk of endometrial CA in PJS reported ≈ 9%
  • 10-18 times >> general population
  • simultaneous multiple gynecological tumors (current case)
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Current Case

  • Simultaneous GYN tumors?

 PJS germline STK11 gene mutation linked to both ?  hyper-estrogenism caused by SCTAT lead to endometrial hyperplasia/ cancer ?

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References

1. Cancer (1982) 50: 1384-1402. Scully RE et al. 2. Am J Pathol (2000)156: 339-345. Connolly DC et al. 3. Gynecol Oncol (2004) 92(1):337-42. Mangili G et al. 4. Am J Surg Pathol (2005) 29(2):157-66. Murray SK(1), Clement PB, Young RH. 5. Int J Gynecol Cancer (2009) 19: 1591-1594. Clements A et al. 6. Eur J Gynaecol Oncol (2011);32(4):452-4. Kondi-Pafiti A et al.

  • 7. Onco Lett (2013); 6:1184-1188. Banno K et al.
  • 8. Int J Clin Exp Pathol (2014);7(7):4448-4453. Zhou F et al.
  • 9. BMC Cancer (2015) 15:270. Qian et al.
  • 10. Zhonghua Bing Li Xue Za Zhi (2016|) 8;45(5):297-301. DOI:

10.3760/cma.j.issn.0529-5807.2016.05.003. Chinese. Sun YH et al.

  • 11. Histol Histopathol (2009 )24(2):149-55. DOI: 10.14670/HH-24.149. Wani

Y et al.

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Thank you

Department of Pathology, University of Jordan Amman, Jordan