Case Presentation of Retroperitoneal Mass

2. Case Presentation ofRetroperitoneal Mass By : Dr. Khalid Jamal Hamdi

3. History MRN:517400 (Hera Hospital) Saad is 43 y/o saudi male patient Presented to ER Main complaint: left lower quadrant pain x 2days associated with Lower back pain

4. History • HPI: not known to have any medical illness before presented to ER C/O LLQ pain x 2 days ,which started gradually, dull aching ,mild ,radiating to the back ,not aggrevated , relieved by NSAID. The patient had a previous similar attacks during the last 6 months but he didn’t seek medical advise

5. History • Review of systems: No H/o : Dysuria ,nausea ,vomiting ,change in Bowel habit, fever. No H/o : trauma other review of systems were unremarkable Past M Hx: unremarkable. Past surgical Hx: appendectomy 14 y ago. Social Hx :Smoker 2 packs /day married ,had 5 kids Allergy: No non allergies to food or medication

6. Examination • Vitals : HR:60 bpm ,RR: 18 BP:130/90 , T : 36.7 • General: conscious ,alert ,oriented not in pain , No Jaundice or pallor No cyanosis , ( PI: 2/10 ) • Chest : clear ,EAE bilateraly • CVS: S1 + S2 + 0

7. Examination • Abdomen: not distended, • scar for appendectomy Soft,lax , no tenderness , no organomegaly No palpable mass Audiable bowel sound • CNS: Normal

8. Investigations • CBC, Hgb:16 g/dl ,WBC:4.0 ,Plt:220 • Chemistry: Normal • Abdominal U/S :done in a private clinic showshuge echogenic well defined homogenously solid mass (11.7x8.4 cm) at retrovesical pouch (?? Colonic mass)

9. Investigations • CT abdomen &pelvis (triple contrast): shows evidence of well defined soft tissue mass in the pelvic cavity about (10.5x8.5cm) seen at the area behind the urinary bladder more to the left side at the level of rectosigmoid with pedicle toward the sacrum. the mass displace the UB ant. and Compressing the area of rectosigmoid

10. Investigations • CT abdomen &pelvis (triple contrast): Cont’ No bony erosions Other abdominal organs are normal No free fluid • Colonoscopy: Normal study

11. differential diagnosis of retroperitoneal mass ( in general ) • 1. Primary Tumors of Retroperitoneum • a. Sarcomas • b. Neuroendocrine tumors - neurofibroma, ganglioneuroma, Schwannomas, …etc. • c. Lymphomas • d. Diffuse retroperitoneal carcinomas (undifferentiated and metastatic origen) • E. kidney,adrenal and ureteric tumors

12. differential diagnosis of retroperitoneal mass • 2. Retroperitoenal Fibrosis • a. Primary (idiopathic) - about 70% of cases • b. Secondary (listed below) • Drugs - methysergide, methyldopa, ergot alkyloids • Periarteritis - usually with aortic aneurysm • Malignant tumors • Radiational fibrosis • Infection - intrabdominal, gonorrhea, abscess • Lymphangitis • Retroperitoneal Hemorrhage (often after invasive proceedure) • Connective Tissue / Autoimmune Disease – systemic sclerosis, systemic lupus • Granulomatous Disease - tuberculosis, sarcoidosis

13. Management • Plan :Exploratory Lap, total excision of retroperitoneal mass Finding intra operatively: huge retroperitoneal mass approximately 10x10 cm adherent to Lt ureter ,Lt Iliac vessels, presacral fascia

17. Histopathology. • Histological features of benign peripheral nerve sheath tumor, neurilemoma(cellular schwannoma)

18. Schwannomas Schwannomas ( neurilemomas). are usually benign tumors arising from Schwann cells of the peripheral nerve sheath. and belong to the category of neural sheath tumors

19. Schwannomas Most schwannomas are benign, although malignant cases are known to occur, especially when there is association with Von Recklinghausen‘s disease, rarely occur in the retroperitoneum, comprising 0.5% to 5% of all schwannomas.

20. Schwannomas Their usual location is the head, neck, the flexor surfaces of the extremities and the posterior mediastinum or the retroperitoneum They can be found in any nerve trunk, except for ?? , Bastounis E, Asimacopoulos PJ, Picoulis E, Leppaniemi AK, Aggouras D, Papakonstandinou K, Papalambros E: Benign retroperitoneal neural sheath tumors in patients without von Recklinghausen's disease. Scand J Urol Nephrol

21. Schwannomas Except cranial nerves I and II

22. Schwannomas Diagnosis in the retroperitoneal position is difficult, and a large and deeply situated tumor is usually present before patients have any symptoms • symptoms are vague and nonspecific, such as vague abdominal pain and dull ache. • Atypical presentations: are very rare and include flank pain and hematuria, headache, and secondary hypertension and recurrent renal colic pain.

23. Schwannomas gross appearance, schwannomas are usually solitary, well circumscribed, firm, smooth-surfaced tumors Histologically, schwannomas consist of compact cellular lesions (Antoni type A tissue) and loose, hypocellular myxoid lesions with microcystic spaces (Antoni type B tissue)., The hallmark pattern of the benign variants is an alternation of these Antoni A and B areas, with a diffuse positivity for S100 protein in the cytoplasm of the tumor cells.

25. Schwannomas Immunohistochemistry shows , microfilament proteins and S-100 protein, which is the neural protein within the Schwann cell Makes it easy to differentiates schwannomas from neurofibromas,

26. Schwannomas A variant of the typical schwannoma is the "ancient type" or "degenerative neurilemoma" that presents with features of degeneration, cystic changes and hyalinization

27. Schwannomas

28. Schwannomas Malignant degeneration of schwannomas is extremely rare, but when present, they act as high-grade sarcomas with a high likelihood of producing local recurrence and distant metastasis.

29. Schwannomas • Radiological studies are fundamental in the diagnostic evaluation. Computed tomography(CT) scans typically show welldefined low or mixed attenuation with cystic necrotic central areas. Cystic changes occur more commonly in retroperitoneal schwannomas (up to 66%) than in other retroperitoneal tumors.

30. Schwannomas Other degenerative changes, such as calcification, hemorrhage, and hyalinization, can also be present

31. Schwannomas • CT-guided core biopsy and fine needle aspiration have been founded to be unreliable for the diagnosis of retroperitoneal schwannoma. Risk of hemorrhage, infection, and tumor seeding; thus, many authors do not recommend CT-guided biopsy.

32. Schwannomas • Management : surgical resection is the only accurate approach for pathologic evaluation to enable diagnosis of retroperitoneal schwannoma.

33. Schwannomas Recent advances in laparoscopic instruments and skills have made laparoscopy an excellent approach for biopsy and even surgical resection of benign retroperitoneal tumors

34. Schwannomas Even though the best management of retroperitoneal neural sheath tumors is complete excision, considerable controversy exists over negative soft tissue margins.

35. Schwannomas The argument here is that the morbidity associated with resection of adjacent tissue would not be justified in the treatment of a benign lesion prognosis for retroperitoneal benign schwannoma is extremely good

36. Schwannomas • Followup:malignant transformations have been reported, usually associated with von Recklinghausen‘s disease.(*) There are a few reported cases in which metastases occurred after resection of a histologically benign schwannoma.?? Therefore, it is suggested that careful monitoring is necessary after removal of benign retroperitoneal Schwannomas Ohigashi T, Nonaka S, Nakanoma T, Ueno M, Deguchi N. Laparoscopic treatment of retroperitoneal benign schwannoma. Int J Urol

37. Schwannomas Thanks