Plexiform neurofibroma mimicking a pancreatic cystic tumour

Vidaud, Dominique; Mp, Vullierme; Colnot, Nathalie; Hénin, Dominique; Ruszniewski, Philippe; Bédossa, Pierre; Couvelard, Anne

doi:10.1136/gut.2005.074609

Cited by 6 publications

(6 citation statements)

References 4 publications

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“…They show an absence of nodular or fascicular structure and are likely to involve skin. The MRI findings in our case were similar to those described elsewhere 7–11 …”

supporting

confidence: 89%

“…In the current case, the location of the nontender, nonreducible globular swelling at the nape of the neck warranted seeking a clear‐cut diagnosis. Magnetic resonance imaging was used, although the imaging evaluation of superficial neurofibromas has been limited to only a few cases 5–9 . On MR imaging, deep plexiform neurofibromas have a typical target‐like appearance, with central hypointensity and peripheral hyperintensity.…”

mentioning

confidence: 99%

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Solitary Plexiform Neurofibroma(s): Role of Magnetic Resonance Imaging

Sehgal¹,

Srivastava²,

Aggarwal³

et al. 2007

Skinmed

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A 10-year-old boy presented with a painless progressive swelling on the nape of the neck of 8 years' duration. When the child was 1 year old, the swelling appeared as a minute raised skin eruption the size of a pearl at the back of the neck. It was painless and progressive, continuing to increase in size until it reached the size of a walnut. At age 2 years, a minor surgical intervention was undertaken to remove the swelling, but a year later, it recurred at the same site. The size continued to increase until it reached the present size. Examination of the afflicted skin surface showed the presence of a globular, nonreducible swelling measuring 7.5 cm x 5 cm located on the upper posterior portion of the neck. Both the swelling and the skin over it were mobile and nontender. Otherwise, the boy's skin surface was without blemishes. On palpation, the swelling was plexiform, resembling a bag of worms. Magnetic resonance imaging (MRI) was performed on 1.5T MR (Signa, GE Healthcare, Chalfont St Giles, England) using T1 and T2 weighted fast spin echo and short tau inversion recovery sequences in the axial and sagittal planes. The images were marked by a well-defined, homogeneous signal intensity lesion in the subcutaneous plane of the nape of the neck. The lesion appeared isointense in T1W images and hyperintense in T2W images with few flow voids within the lesion. Based on the MRI findings, a diagnosis of superficial plexiform neurofibroma was considered. Results of routine liver and kidney function blood examination tests were within normal limits. Surgical resection was done under general anesthesia. As soon as the skin was excised, the swelling started bleeding profusely, but bleeding stopped almost immediately on the complete excision of the swelling. No cavities were present in the mass, and the underlying structures were normal. The wound was closed with nonabsorbable monofilament nylon suture. The postoperative period was uneventful. In addition to initial excision biopsy, all of the resected tissue material was subjected to serial sectioning for microscopic pathologic examination. Hematoxylin- and eosin-stained sections showed unremarkable epidermis with slight hyperkeratosis. Upper dermis showed circumscribed, lobular proliferation of spindle cells in a loose fibular background distorting much of the dermis and subcutaneous fat. Subcutis showed numerous thin-walled, ectatic blood vessels lined with prominent endothelium. Intervening tissue consisted of wavy bundles of collagen lined with elongated thin cells with tapering and wavy nuclei. The nuclear chromatin was bland without significant polymorphism or raised mitotic activity. A few nerve twigs were also seen within the tumor, which was infiltrating the surrounding fat.

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“…They show an absence of nodular or fascicular structure and are likely to involve skin. The MRI findings in our case were similar to those described elsewhere 7–11 …”

supporting

confidence: 89%

mentioning

confidence: 99%

Solitary Plexiform Neurofibroma(s): Role of Magnetic Resonance Imaging

Sehgal¹,

Srivastava²,

Aggarwal³

et al. 2007

Skinmed

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“…At immunohistochemistry, the tumors express S100. P53 negativity and sparse Ki-67 positivity confirm the benign nature of the tumors (3,4).…”

Section: Solid Neoplasms Neurofibromamentioning

confidence: 78%

Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1—Benign Tumors:From the Radiologic Pathology Archives

Manning¹,

Srivastava²,

Paal³

et al. 2016

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Solid and cystic pancreatic neoplasms are being recognized more frequently with increasing utilization and spatial resolution of modern imaging techniques. In addition to the more common primary pancreatic solid (ductal adenocarcinoma) and cystic neoplasms of epithelial origin, nonepithelial neoplasms of the pancreas may appear as well-defined solid or cystic neoplasms. Most of these lesions have characteristic imaging features, such as a well-defined border, which allows differentiation from ductal adenocarcinoma. Solid masses include neurofibroma, ganglioneuroma, leiomyoma, lipoma, and perivascular epithelioid cell tumor (PEComa). Schwannomas and desmoid tumors can be solid or cystic. Cystic tumors include mature cystic teratoma and lymphangioma. Lipoma, PEComa, and mature cystic teratoma can contain fat, and ganglioneuroma and mature cystic teratoma may contain calcification. Although these unusual benign neoplasms are rare, the radiologist should at least consider them in the differential diagnosis of well-defined lesions of the pancreas. The goal of this comprehensive review is to improve understanding of these rare primary pancreatic mesenchymal tumors.

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“…Mesenchymal spindle cell neoplasms are one of the most challenging aspects of cytology with considerable overlap in morphology and ICC. Gastrointestinal stromal tumour (GIST), leiomyoma, ganglioneuroma,schwannoma, desmoids fibromatosis, solitary fibrous tumour (SFT), inflammatory myofibroblastic tumour, low‐grade myofibroblastic sarcoma and neurofibroma are all reported in the pancreas. These lesions show tightly cohesive cellular fragments of spindle cells variably arranged in fascicular pattern associated with metachromatic matrix material.…”

Section: Discussionmentioning

confidence: 99%