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DermAtlas: Histology - neurofibroma, plexiform
© 2001-2009, DermAtlas
Image Name: plexiform_neurofibroma_1_090401   File Type: jpg
Diagnosis: NEUROFIBROMA, PLEXIFORM /
NEUROFIBROMATOSIS
  Category: neoplasm, neural /
neoplasm, benign /
genodermatosis/genetic disorder
Body Site: thigh   Age: 19 years
Contributor: Greg Hosler, MD, PhD    
Description: At scanning magnification, there are discrete well-circumscribed collections of spindle cells. The interval dermis has loose collagen. The epidermis is uninvolved.
Comments: This young man with NF-1 mutation developed a slow growing mass on his thigh. The mass was ill-defined and measured several centimeters in greatest dimension. There was a reported "bag of worms" quality to the mass, as is often the case with plexiform neurofibromas. This gross description is due to the tumor snaking around as a tube in three dimensions. When this is cut on a two dimensional slide, it appears as separate circumscribed nodules, as in this case. The tumo is made up of multiple cell types, the cells normally observed in the nerve sheath - mast cells, fibroblasts, Schwann cells, etc. The plexiform variant is virtually pathognomonic neurofibromatosis type 1.
Related Images: plexiform_neurofibroma_2_090401 

DermAtlas: Histology - neurofibroma, plexiform
© 2001-2009, DermAtlas
Image Name: plexiform_neurofibroma_2_090401   File Type: jpg
Diagnosis: NEUROFIBROMA, PLEXIFORM /
NEUROFIBROMATOSIS
  Category: neoplasm, neural /
neoplasm, benign /
genodermatosis/genetic disorder
Body Site: thigh   Age: 19 years
Contributor: Greg Hosler, MD, PhD    
Description: The tumor is made up of mulltiple cell types, including fiboblasts, Schwann cells and interspersed mast cells. These appear well-contained within the nerve sheath. Thre is no significant cytologic atypia or mitotic activity. The stroma is fibromyxoid.
Comments: This young man with NF-1 mutation developed a slow growing mass on his thigh. The mass was ill-defined and measured several centimeters in greatest dimension. There was a reported "bag of worms" quality to the mass, as is often the case with plexiform neurofibromas. This gross description is due to the tumor snaking around as a tube in three dimensions. When this is cut on a two dimensional slide, it appears as separate circumscribed nodules, as in this case. The tumo is made up of multiple cell types, the cells normally observed in the nerve sheath - mast cells, fibroblasts, Schwann cells, etc. The plexiform variant is virtually pathognomonic neurofibromatosis type 1.
Related Images: plexiform_neurofibroma_1_090401 

DermAtlas: Histology - perineurioma
© 2001-2009, DermAtlas
Image Name: perineurioma_1_071101   File Type: jpg
Diagnosis: PERINEURIOMA   Category: neoplasm, neural /
neoplasm, benign
Body Site: finger   Age: 55 years
Contributor: Greg Hosler, MD, PhD    
Description: There is a nodular sclerotic lesion within the superficial dermis. The cells form whorls and a vaguely storiform pattern, with infiltrative borders. Individual tumor cells are plump and spindled, with mild amounts of pink cytoplasm.
Comments: This was a typical presentation of a perineurioma and was submitted as a cyst. A perineurioma is an unusual tumor, derived from the perineurium. The histologic differential diagnosis includes a storiform collagenoma or sclerotic fibroma. This tumor is usually more cellular than a sclerotic fibroma, but an EMA immunostain will easily distinguish the two.
Related Images: perineurioma_2_071101 

DermAtlas: Histology - perineurioma
© 2001-2009, DermAtlas
Image Name: perineurioma_2_071101   File Type: jpg
Diagnosis: PERINEURIOMA   Category: neoplasm, neural /
neoplasm, benign
Body Site: finger   Age: 55 years
Contributor: Greg Hosler, MD, PhD    
Description: The tumor is positive for epithelial membrane antigen (EMA).
Comments: This was a typical presentation of a perineurioma and was submitted as a cyst. A perineurioma is an unusual tumor, derived from the perineurium. The histologic differential diagnosis includes a storiform collagenoma or sclerotic fibroma. This tumor is usually more cellular than a sclerotic fibroma, but an EMA immunostain will easily distinguish the two.
Related Images: perineurioma_1_071101 

DermAtlas: Histology - neurofibroma
© 2001-2009, DermAtlas
Image Name: neurofibroma_1_070501   File Type: jpg
Diagnosis: NEUROFIBROMA   Category: neoplasm, neural /
neoplasm, benign
Body Site: chest   Age: 74 years
Contributor: Greg Hosler, MD, PhD    
Description: There is a fairly well-demarcated but unencapsulated spindle cell tumor within the dremis. The epidermis is uninvolved. The spindle cells are monomorphic and imperceptibly blend into the surrounding dermis. The lesion is slightly more pink than the dermal collagen.
Comments: This is a very typical example of a common lesion. The histologic differential diagnosis includes a neuritized nevus and perhaps other soft tissue tumors such as a dermatofibroma or neuroma. The lesion contains a mixture of axons, mast cells, and nerve sheath cells (fibroblasts and Schwann cells). These will stain positive for S-100 and neurofilament (scattered twigs) if needed.
Related Images: neurofibroma_2_070501 

DermAtlas: Histology - neurofibroma
© 2001-2009, DermAtlas
Image Name: neurofibroma_2_070501   File Type: jpg
Diagnosis: NEUROFIBROMA   Category: neoplasm, neural /
neoplasm, benign
Body Site: chest   Age: 74 years
Contributor: Greg Hosler, MD, PhD    
Description: A higher power shows the very characteristic round, thin-walled vessels and the mixed nature of the tumor cells. One can identify fibroblast-like cells, Schwann cells, and scattered purple mast cells. There is no cytologic atypia or mitotic activity.
Comments: This is a very typical example of a common lesion. The histologic differential diagnosis includes a neuritized nevus and perhaps other soft tissue tumors such as a dermatofibroma or neuroma. The lesion contains a mixture of axons, mast cells, and nerve sheath cells (fibroblasts and Schwann cells). These will stain positive for S-100 and neurofilament (scattered twigs) if needed.
Related Images: neurofibroma_1_070501 

DermAtlas: Histology - carcinoma, Merkel cell
© 2001-2009, DermAtlas
Image Name: merkel_cell_carcinoma_1_060220   File Type: jpg
Diagnosis: CARCINOMA, MERKEL CELL   Category: neoplasm, neural /
neoplasm, malignant
Body Site: forearm   Age: 79 years
Contributor: Greg Hosler, MD, PhD    
Description: Histologic sections of skin show a basophilic cellular neoplasm infiltrating throughout the dermis. Several nests are present within the papillary dermis, but the deeper component is poorly defined, showing marked crush artifact.
Comments: This is a fairly typical example of primary neuroendocrine carcinoma of the skin (Merkel cell carcinoma). This specimen was submitted as "rule out metastasis, lymphoma, etc", likely due to its juicy violaceous character. The histology here is classic. Crush artifact is often seen with Merkel cell carcinoma but can also be seen with other entities in the differential: lymphoma, leukemia cutis, and small cell carcinoma. Although immunohistochemical analysis is advised, the histologic appearance of Merkel cell carcinoma is quite distinctive. The cells often mold against each other, giving a sense of cohesion. The nuclei are steel gray with very finely stippled chromatin, unlike that seen in lymphoma. Merkel cell carcinoma should stain with neural markers, such as neuron specific enolase (NSE) and neurofilament, as in this case. Chromogranin and synaptophysin are also positive. Keratin stains (CK20 is classic, but others like CAM5.2 work too) show a characteristic perinuclear "dot-like" pattern. A negative TTF-1 may be the only evidence against a metastatic small cell carcinoma.
Related Images: All related Images  merkel_cell_carcinoma_3_060220  merkel_cell_carcinoma_2_060220 

DermAtlas: Histology - carcinoma, Merkel cell
© 2001-2009, DermAtlas
Image Name: merkel_cell_carcinoma_2_060220   File Type: jpg
Diagnosis: CARCINOMA, MERKEL CELL   Category: neoplasm, neural /
neoplasm, malignant
Body Site: forearm   Age: 79 years
Contributor: Greg Hosler, MD, PhD    
Description: At higher power of the same lesion, an aggregate of malignant cells show the characteristic molding pattern. The nuclei are steel gray with very fine, stippled chromatin. Background tumor cells show marked crush artifact. Necrosis and high mitotic activity are the norm.
Comments: This is a fairly typical example of primary neuroendocrine carcinoma of the skin (Merkel cell carcinoma). This specimen was submitted as "rule out metastasis, lymphoma, etc", likely due to its juicy violaceous character. The histology here is classic. Crush artifact is often seen with Merkel cell carcinoma but can also be seen with other entities in the differential: lymphoma, leukemia cutis, and small cell carcinoma. Although immunohistochemical analysis is advised, the histologic appearance of Merkel cell carcinoma is quite distinctive. The cells often mold against each other, giving a sense of cohesion. The nuclei are steel gray with very finely stippled chromatin, unlike that seen in lymphoma. Merkel cell carcinoma should stain with neural markers, such as neuron specific enolase (NSE) and neurofilament, as in this case. Chromogranin and synaptophysin are also positive. Keratin stains (CK20 is classic, but others like CAM5.2 work too) show a characteristic perinuclear "dot-like" pattern. A negative TTF-1 may be the only evidence against a metastatic small cell carcinoma.
Related Images: All related Images  merkel_cell_carcinoma_3_060220  merkel_cell_carcinoma_1_060220 

DermAtlas: Histology - neurothekeoma
© 2001-2009, DermAtlas
Image Name: neurothekeoma_1_060220   File Type: jpg
Diagnosis: NEUROTHEKEOMA   Category: neoplasm, neural /
neoplasm, benign
Body Site: leg   Age: 39 years
Contributor: Greg Hosler, MD, PhD    
Description: Histologic sections of skin show a lobular spindle cell lesion within the dermis. The overlying epidermis shows mild acanthosis and papillomatosis. The spindle cells are in variably sized lobules with a mucinous/myxoid stroma and have thick fibrous septae. An inflammatory response is minimal.
Comments: This is a classic histologic example of a myxoid neurothekeoma, or nerve sheath myxoma. Clinically, they typically present on the head, neck, and upper extremities of middle age women, and therefore the location in this example is somewhat unusual. In difficult cases, the differential diagnosis may include cutaneous myxoma, mucus/myxoid cyst, myxoid neurofibroma, and mucinous adenocarcinoma. The lobular nature of this one is unmistakable. These lesions are usually S100 positive and GFAP positive, as in this case. They also stain with neuron specific enolase (NSE) and CD57. The cellular neurothekeomas are odd, expressing actin and only variably staining with neural markers.
Related Images: All related Images  neurothekeoma_4_060220  neurothekeoma_3_060220  neurothekeoma_2_060220 

DermAtlas: Histology - neurothekeoma
© 2001-2009, DermAtlas
Image Name: neurothekeoma_2_060220   File Type: jpg
Diagnosis: NEUROTHEKEOMA   Category: neoplasm, neural /
neoplasm, benign
Body Site: leg   Age: 39 years
Contributor: Greg Hosler, MD, PhD    
Description: A different portion of the same lesion shows similar features. Variably sized lobules of spindle cells within a myxoid stroma are separated by thick fibrous septae.
Comments: This is a classic histologic example of a myxoid neurothekeoma, or nerve sheath myxoma. Clinically, they typically present on the head, neck, and upper extremities of middle age women, and therefore the location in this example is somewhat unusual. In difficult cases, the differential diagnosis may include cutaneous myxoma, mucus/myxoid cyst, myxoid neurofibroma, and mucinous adenocarcinoma. The lobular nature of this one is unmistakable. These lesions are usually S100 positive and GFAP positive, as in this case. They also stain with neuron specific enolase (NSE) and CD57. The cellular neurothekeomas are odd, expressing actin and only variably staining with neural markers.
Related Images: All related Images  neurothekeoma_4_060220  neurothekeoma_3_060220  neurothekeoma_1_060220 

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Bernard A. Cohen, MD, Christoph U. Lehmann, MD

DermAtlas was last updated: Oct-28-2009
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