Dendritic cell neurofibroma with pseudorosettes: A report of 18 cases of a distinct and hitherto unrecognized neurofibroma variant

Michal Michal, Julie C. Fanburg-Smith, Thomas Mentzel, Heinz Kutzner, Luis Requena, Michal Zamecnik, Markku Miettinen

Research output: Contribution to journalArticle

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Abstract

The authors present 18 cases of a hitherto unrecognized variant of cutaneous neurofibroma. The tumors presented in adults (10 occurred in men and eight occurred in women) as a solitary, well-circumscribed, superficial lesion located in the dermis measuring 3 to 17 mm (mean size, 6.2 mm). The tumors formed oval-shaped masses that ran perpendicular to the epidermis. In the deep part of the tumor there was multinodular arrangement with two types of cells: Type I cells were small, dark, lymphocyte-like cells with a slightly irregular nucleus and inconspicuous cytoplasm. Type II cells were larger, with palestaining vesicular nuclei, with frequent invaginations and intranuclear inclusions, and had copious clear eosinophilic cytoplasm that formed a stellate growth pattern, which was poorly visible on hematoxylin and eosin staining. Type I cells were grouped concentrically around type II cells and formed pseudorosettes. Most of the type I and type II cells were S-100 protein and CD57 positive, and various proportions of both cell types were CD56 and PGP9.5 positive. All cells were chromogranin A, synaptophysin, glial fibrillary acidic protein, cytokeratins, CD1a, CD21, CD31, α-smooth muscle actin, muscle-specific actin, desmin, and HMB-45 negative. CD34 stained intralesional fibroblasts. Antibody to epithelial membrane antigen stained only the perineurium around the tumor masses, suggesting that the tumors arose inside the nerve sheath. No signs of neurosecretory granules were present at ultrastructural level. None of the lesions recurred and none metastasized over a mean follow-up of 8.1 years.

Original languageEnglish (US)
Pages (from-to)587-594
Number of pages8
JournalAmerican Journal of Surgical Pathology
Volume25
Issue number5
DOIs
StatePublished - May 21 2001

Fingerprint

Neurofibroma
Dendritic Cells
Neoplasms
Actins
Cytoplasm
Intranuclear Inclusion Bodies
Chromogranin A
Mucin-1
Synaptophysin
Desmin
S100 Proteins
Glial Fibrillary Acidic Protein
Hematoxylin
Eosine Yellowish-(YS)
Dermis
Keratins
Peripheral Nerves
Epidermis
Smooth Muscle
Fibroblasts

All Science Journal Classification (ASJC) codes

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Michal, Michal ; Fanburg-Smith, Julie C. ; Mentzel, Thomas ; Kutzner, Heinz ; Requena, Luis ; Zamecnik, Michal ; Miettinen, Markku. / Dendritic cell neurofibroma with pseudorosettes : A report of 18 cases of a distinct and hitherto unrecognized neurofibroma variant. In: American Journal of Surgical Pathology. 2001 ; Vol. 25, No. 5. pp. 587-594.
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abstract = "The authors present 18 cases of a hitherto unrecognized variant of cutaneous neurofibroma. The tumors presented in adults (10 occurred in men and eight occurred in women) as a solitary, well-circumscribed, superficial lesion located in the dermis measuring 3 to 17 mm (mean size, 6.2 mm). The tumors formed oval-shaped masses that ran perpendicular to the epidermis. In the deep part of the tumor there was multinodular arrangement with two types of cells: Type I cells were small, dark, lymphocyte-like cells with a slightly irregular nucleus and inconspicuous cytoplasm. Type II cells were larger, with palestaining vesicular nuclei, with frequent invaginations and intranuclear inclusions, and had copious clear eosinophilic cytoplasm that formed a stellate growth pattern, which was poorly visible on hematoxylin and eosin staining. Type I cells were grouped concentrically around type II cells and formed pseudorosettes. Most of the type I and type II cells were S-100 protein and CD57 positive, and various proportions of both cell types were CD56 and PGP9.5 positive. All cells were chromogranin A, synaptophysin, glial fibrillary acidic protein, cytokeratins, CD1a, CD21, CD31, α-smooth muscle actin, muscle-specific actin, desmin, and HMB-45 negative. CD34 stained intralesional fibroblasts. Antibody to epithelial membrane antigen stained only the perineurium around the tumor masses, suggesting that the tumors arose inside the nerve sheath. No signs of neurosecretory granules were present at ultrastructural level. None of the lesions recurred and none metastasized over a mean follow-up of 8.1 years.",
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Dendritic cell neurofibroma with pseudorosettes : A report of 18 cases of a distinct and hitherto unrecognized neurofibroma variant. / Michal, Michal; Fanburg-Smith, Julie C.; Mentzel, Thomas; Kutzner, Heinz; Requena, Luis; Zamecnik, Michal; Miettinen, Markku.

In: American Journal of Surgical Pathology, Vol. 25, No. 5, 21.05.2001, p. 587-594.

Research output: Contribution to journalArticle

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AU - Michal, Michal

AU - Fanburg-Smith, Julie C.

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AU - Zamecnik, Michal

AU - Miettinen, Markku

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N2 - The authors present 18 cases of a hitherto unrecognized variant of cutaneous neurofibroma. The tumors presented in adults (10 occurred in men and eight occurred in women) as a solitary, well-circumscribed, superficial lesion located in the dermis measuring 3 to 17 mm (mean size, 6.2 mm). The tumors formed oval-shaped masses that ran perpendicular to the epidermis. In the deep part of the tumor there was multinodular arrangement with two types of cells: Type I cells were small, dark, lymphocyte-like cells with a slightly irregular nucleus and inconspicuous cytoplasm. Type II cells were larger, with palestaining vesicular nuclei, with frequent invaginations and intranuclear inclusions, and had copious clear eosinophilic cytoplasm that formed a stellate growth pattern, which was poorly visible on hematoxylin and eosin staining. Type I cells were grouped concentrically around type II cells and formed pseudorosettes. Most of the type I and type II cells were S-100 protein and CD57 positive, and various proportions of both cell types were CD56 and PGP9.5 positive. All cells were chromogranin A, synaptophysin, glial fibrillary acidic protein, cytokeratins, CD1a, CD21, CD31, α-smooth muscle actin, muscle-specific actin, desmin, and HMB-45 negative. CD34 stained intralesional fibroblasts. Antibody to epithelial membrane antigen stained only the perineurium around the tumor masses, suggesting that the tumors arose inside the nerve sheath. No signs of neurosecretory granules were present at ultrastructural level. None of the lesions recurred and none metastasized over a mean follow-up of 8.1 years.

AB - The authors present 18 cases of a hitherto unrecognized variant of cutaneous neurofibroma. The tumors presented in adults (10 occurred in men and eight occurred in women) as a solitary, well-circumscribed, superficial lesion located in the dermis measuring 3 to 17 mm (mean size, 6.2 mm). The tumors formed oval-shaped masses that ran perpendicular to the epidermis. In the deep part of the tumor there was multinodular arrangement with two types of cells: Type I cells were small, dark, lymphocyte-like cells with a slightly irregular nucleus and inconspicuous cytoplasm. Type II cells were larger, with palestaining vesicular nuclei, with frequent invaginations and intranuclear inclusions, and had copious clear eosinophilic cytoplasm that formed a stellate growth pattern, which was poorly visible on hematoxylin and eosin staining. Type I cells were grouped concentrically around type II cells and formed pseudorosettes. Most of the type I and type II cells were S-100 protein and CD57 positive, and various proportions of both cell types were CD56 and PGP9.5 positive. All cells were chromogranin A, synaptophysin, glial fibrillary acidic protein, cytokeratins, CD1a, CD21, CD31, α-smooth muscle actin, muscle-specific actin, desmin, and HMB-45 negative. CD34 stained intralesional fibroblasts. Antibody to epithelial membrane antigen stained only the perineurium around the tumor masses, suggesting that the tumors arose inside the nerve sheath. No signs of neurosecretory granules were present at ultrastructural level. None of the lesions recurred and none metastasized over a mean follow-up of 8.1 years.

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