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Case Presentation of Retroperitoneal Mass. By : Dr. Khalid Jamal Hamdi. 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. History.

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case presentation of retroperitoneal mass

Case Presentation ofRetroperitoneal Mass

By : Dr. Khalid Jamal Hamdi

history
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

history1
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

history2
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

examination
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
examination1
Examination
  • Abdomen: not distended,
  • scar for appendectomy

Soft,lax , no tenderness , no organomegaly

No palpable mass

Audiable bowel sound

  • CNS: Normal
investigations
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)

investigations1
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

investigations2
Investigations
  • CT abdomen &pelvis (triple contrast):

Cont’

No bony erosions

Other abdominal organs are normal

No free fluid

  • Colonoscopy: Normal study
differential diagnosis of retroperitoneal mass in general
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
differential diagnosis of retroperitoneal mass
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
management
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

histopathology
Histopathology.
  • Histological features of benign peripheral nerve sheath tumor,

neurilemoma(cellular schwannoma)

schwannomas
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

schwannomas1
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.

schwannomas2
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

schwannomas3
Schwannomas

Except cranial nerves I and II

schwannomas4
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.

schwannomas5
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.

schwannomas6
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,

schwannomas7
Schwannomas

A variant of the typical schwannoma is the "ancient type"

or "degenerative neurilemoma" that presents with features

of degeneration, cystic changes and hyalinization

schwannomas9
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.

schwannomas10
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.

schwannomas11
Schwannomas

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

schwannomas12
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.

schwannomas13
Schwannomas
  • Management :

surgical resection is the only accurate approach

for pathologic evaluation to enable diagnosis of retroperitoneal

schwannoma.

schwannomas14
Schwannomas

Recent advances in laparoscopic instruments

and skills have made laparoscopy an excellent

approach for biopsy and even surgical resection of benign retroperitoneal tumors

schwannomas15
Schwannomas

Even though the best management of retroperitoneal

neural sheath tumors is complete excision,

considerable

controversy exists over negative soft tissue margins.

schwannomas16
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

schwannomas17
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