PMC9458937_01
Individuals who have neurofibromatosis type 1 NF1 may exhibit a variety of cutaneous characteristics, including (A), the growth of nerve sheath tumors (neurofibromas)is a prominent hallmark of NF1. Neurofibromas can develop as isolated nodules or as cutaneous neurofibromas (B). pigmentary feature of NF1 patient (cafe-au-lait macules) on the back of the patient as (arrow).
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F1
At the initial examination, significant cafe au lait spots and freckling were found over the patient's body, but the mass filling the left abdominal quadrant was inaccessible (Figure 1).
Abdominal MRI shows an irregular retroperitoneal mass under the pancreatic body and the pancreatic uncinate process is pushed forward to the right. The mass had about 3.8 cm x 5.7 cm x 2.8 cm in size, which has unclear boundary with some surrounding intestinal loops (A,B). Coronal T2-weighted image depicts high signal tumor; (C) Transverse Diffusion-weighted image shows obvious high signal tumor; (D-F) enhancement MRI scan on arterial, venous and excretion stages show uneven and obvious enhancement.
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F2
T1W image illustrates a low signal mass, the T2-weighted image indicates a high-signal tumor; and the diffusion-weighted displays an obvious high-signal tumor; additionally, enhanced MRI scans of the arterial, venous, and excretion phases clearly show uneven and noticeable enhancement (Figure 2). There were no gastrointestinal problems in our case. as well as the tumor's location based on imaging results led to the establishment of a primary diagnosis of retroperitoneal neurofibroma.
(A) Trocars configuration for left retroperitoneal laparoscopic approach; [trocar a] is inserted via 2 cm skin incision made below the 12th rib, anterior to the Sacro-spinal muscle, on the posterior axillary line and the skin incision is sutured to fix the trocar, [trocar b] A 10 mm camera trocar is inserted two fingers breadths above the iliac crest on midaxillary line; [trocar c] is inserted at the subcostal margin on anterior axillary line (B). Extracted tumor mass specimen (C). Postoperative drain in retroperitoneal space.
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F3
Minimally invasive surgery was performed using a three-port retroperitoneal approach (Figure 3).
Histological sample. (A) Low power H & E Showing spindle shaped tumor cells arranged in fascicles and bundles. for tumor (B). Higher power view showing the spindle appearance of tumor cells. IHC positivity for SDH-B (D). IHC positivity for CD 34 (E). IHC positivity for DOG1 (F). IHC positivity for CD117 (G). IHC negativity for desmin (H). IHC negativity for S-100. H & E, hematoxylin and eosin stain; IHC, immunohistochemistry of tumor cells (200x).
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F4
Histopathologic investigations revealed that the resected mass was a composite of that CD117(+), CD34(+), desmin(-), DOG-1(+), Ki-67(+5%), S-100(-), SDH-B(+), and SMA(-), all of which are consistent with GIST tumor (Figure 4).