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The Dermatopathologist

The Dermatopathologist: What Is This Nodule?

May 2017

Case 1.
A 49-year-old woman with a past medical history of immunosuppression presented with a nodule on the right lower nasofacial sulcus. (Figures A-E).

    a. Drug-induced follicular dystrophy
    b. Virus-associated trichodysplasia spinulosa
    c. Keratosis pilaris
    d. Lichen spinulosis


Case 2.
A 30-year-old woman presented with a subcutaneous mass of the right thigh. (Figures A-F)

    a. Leiomyoma
    b. Inflammatory myofibroblastic tumor
    c. Nodular fasciitis
    d. Myofibroma


Case 3.
A 20-year-old man presented with a nodule on the thigh. (Figures A-F)

    a. Angiosracoma
    b. Kaposi sarcoma
    c. Aneurysmal/angiomatoid fibrous histiocytoma
    d. Hemangioma


Case 4.
A 30-year-old woman presented with a cough, night sweats, and numerous ulcerated nodules of the bilateral arms. (Figures A-F)

    a. Blastomycosis
    b. Cryptococcosis
    c. Leishmaniasis
    d. Histoplasmosis

 

To find out the quiz answers, go to Page 2.

 

QUIZ ANSWERS

Case 1 — B

Virus-Associated Trichodysplasia Spinulosa
The findings are highly distinctive for a unique folliculocentric viral syndrome seen in the setting of immunosuppression, mainly in the context of patients who are organ transplant recipients and/or on regimens of immunosuppressive drug therapy. The histopathology is very typical for trichodysplasia spinulosa, which is thought to be caused by a polyomavirus. Specifically, it is the trichodysplasia spinulosa polyomavirus 8.

The main abnormality is regarding the organization of the hair follicles, whereby there is an unusual hyperproliferation of the inner root sheath demonstrating irregular eosinophilic trichohyalin granules compatible with intracellular viral particles and not surprisingly there are significant dystrophic changes in the hair shaft.


Case 2 — D

Myofibroma
The findings are those of a soft tissue neoplasm which falls under the appellation of a solitary myofibroma. The lesion appears to exhibit an intravascular localization. The cardinal hallmarks of this benign soft tissue neoplasm are well exemplified by this case and include a dichotomous composition characterized by smooth muscle myomatous nodules with interposed hemangiopericytomatous-like foci. Histologically these lesions invariably exhibit a very characteristic biphasic pattern with whorled areas of spindled cells showing hyalinization and chondroid-like alteration of the background matrix with more interposed cellular spindled hypervascular hemangiopericytomatous areas. Phenotypically the myomatous nodules are desmin negative, although they do express smooth muscle actin and calponin.


Case 3 — C

Aneurysmal/Angiomatoid Fibrous Histiocytoma
The findings are characteristic for a rare form of fibrous histiocytoma representing angiomatoid fibrous histiocytoma. This neoplasm is a low-grade mesenchymal tumor of uncertain differentiation typically occurring in children and young adults on the extremities. Its morphologic hallmarks include a multinodular proliferation of spindled to ovoid cells associated with pseudoangiomatoid spaces. Low-grade atypia can be seen and indeed this particular case does exhibit random atypia of the neoplastic cell populace somewhat analogous to atypical fibrous histiocytoma. The neoplastic cells show focal staining for desmin in at least 40% of cases. In this case, weak cytoplasmic immunoreactivity of the neoplastic cells for desmin was noted. These tumors can recur; the metastatic rate is less than 1%, although it has been documented.


Case 4 — A

Blastomycosis
The findings are diagnostic of blastomycosis. A fairly robust neutrophilic and granulomatous response in association with the fungal elements was identified. The fungal forms are spherical with a thick refractile cell wall. They are multinucleate and have a broad-based budding.

 

Dr Magro is the director of dermatopathology at Weill Cornell Medicine in New York, NY. For more information, please visit www.weillcornelldermpath.com.

Disclosure: The author reports no relevant financial relationships.

Case 1.
A 49-year-old woman with a past medical history of immunosuppression presented with a nodule on the right lower nasofacial sulcus. (Figures A-E).

    a. Drug-induced follicular dystrophy
    b. Virus-associated trichodysplasia spinulosa
    c. Keratosis pilaris
    d. Lichen spinulosis


Case 2.
A 30-year-old woman presented with a subcutaneous mass of the right thigh. (Figures A-F)

    a. Leiomyoma
    b. Inflammatory myofibroblastic tumor
    c. Nodular fasciitis
    d. Myofibroma


Case 3.
A 20-year-old man presented with a nodule on the thigh. (Figures A-F)

    a. Angiosracoma
    b. Kaposi sarcoma
    c. Aneurysmal/angiomatoid fibrous histiocytoma
    d. Hemangioma


Case 4.
A 30-year-old woman presented with a cough, night sweats, and numerous ulcerated nodules of the bilateral arms. (Figures A-F)

    a. Blastomycosis
    b. Cryptococcosis
    c. Leishmaniasis
    d. Histoplasmosis

 

 

QUIZ ANSWERS

Case 1 — B

Virus-Associated Trichodysplasia Spinulosa
The findings are highly distinctive for a unique folliculocentric viral syndrome seen in the setting of immunosuppression, mainly in the context of patients who are organ transplant recipients and/or on regimens of immunosuppressive drug therapy. The histopathology is very typical for trichodysplasia spinulosa, which is thought to be caused by a polyomavirus. Specifically, it is the trichodysplasia spinulosa polyomavirus 8.

The main abnormality is regarding the organization of the hair follicles, whereby there is an unusual hyperproliferation of the inner root sheath demonstrating irregular eosinophilic trichohyalin granules compatible with intracellular viral particles and not surprisingly there are significant dystrophic changes in the hair shaft.


Case 2 — D

Myofibroma
The findings are those of a soft tissue neoplasm which falls under the appellation of a solitary myofibroma. The lesion appears to exhibit an intravascular localization. The cardinal hallmarks of this benign soft tissue neoplasm are well exemplified by this case and include a dichotomous composition characterized by smooth muscle myomatous nodules with interposed hemangiopericytomatous-like foci. Histologically these lesions invariably exhibit a very characteristic biphasic pattern with whorled areas of spindled cells showing hyalinization and chondroid-like alteration of the background matrix with more interposed cellular spindled hypervascular hemangiopericytomatous areas. Phenotypically the myomatous nodules are desmin negative, although they do express smooth muscle actin and calponin.


Case 3 — C

Aneurysmal/Angiomatoid Fibrous Histiocytoma
The findings are characteristic for a rare form of fibrous histiocytoma representing angiomatoid fibrous histiocytoma. This neoplasm is a low-grade mesenchymal tumor of uncertain differentiation typically occurring in children and young adults on the extremities. Its morphologic hallmarks include a multinodular proliferation of spindled to ovoid cells associated with pseudoangiomatoid spaces. Low-grade atypia can be seen and indeed this particular case does exhibit random atypia of the neoplastic cell populace somewhat analogous to atypical fibrous histiocytoma. The neoplastic cells show focal staining for desmin in at least 40% of cases. In this case, weak cytoplasmic immunoreactivity of the neoplastic cells for desmin was noted. These tumors can recur; the metastatic rate is less than 1%, although it has been documented.


Case 4 — A

Blastomycosis
The findings are diagnostic of blastomycosis. A fairly robust neutrophilic and granulomatous response in association with the fungal elements was identified. The fungal forms are spherical with a thick refractile cell wall. They are multinucleate and have a broad-based budding.

 

Dr Magro is the director of dermatopathology at Weill Cornell Medicine in New York, NY. For more information, please visit www.weillcornelldermpath.com.

Disclosure: The author reports no relevant financial relationships.

Case 1.
A 49-year-old woman with a past medical history of immunosuppression presented with a nodule on the right lower nasofacial sulcus. (Figures A-E).

    a. Drug-induced follicular dystrophy
    b. Virus-associated trichodysplasia spinulosa
    c. Keratosis pilaris
    d. Lichen spinulosis


Case 2.
A 30-year-old woman presented with a subcutaneous mass of the right thigh. (Figures A-F)

    a. Leiomyoma
    b. Inflammatory myofibroblastic tumor
    c. Nodular fasciitis
    d. Myofibroma


Case 3.
A 20-year-old man presented with a nodule on the thigh. (Figures A-F)

    a. Angiosracoma
    b. Kaposi sarcoma
    c. Aneurysmal/angiomatoid fibrous histiocytoma
    d. Hemangioma


Case 4.
A 30-year-old woman presented with a cough, night sweats, and numerous ulcerated nodules of the bilateral arms. (Figures A-F)

    a. Blastomycosis
    b. Cryptococcosis
    c. Leishmaniasis
    d. Histoplasmosis

 

,

Case 1.
A 49-year-old woman with a past medical history of immunosuppression presented with a nodule on the right lower nasofacial sulcus. (Figures A-E).

    a. Drug-induced follicular dystrophy
    b. Virus-associated trichodysplasia spinulosa
    c. Keratosis pilaris
    d. Lichen spinulosis


Case 2.
A 30-year-old woman presented with a subcutaneous mass of the right thigh. (Figures A-F)

    a. Leiomyoma
    b. Inflammatory myofibroblastic tumor
    c. Nodular fasciitis
    d. Myofibroma


Case 3.
A 20-year-old man presented with a nodule on the thigh. (Figures A-F)

    a. Angiosracoma
    b. Kaposi sarcoma
    c. Aneurysmal/angiomatoid fibrous histiocytoma
    d. Hemangioma


Case 4.
A 30-year-old woman presented with a cough, night sweats, and numerous ulcerated nodules of the bilateral arms. (Figures A-F)

    a. Blastomycosis
    b. Cryptococcosis
    c. Leishmaniasis
    d. Histoplasmosis

 

To find out the quiz answers, go to Page 2.

 

QUIZ ANSWERS

Case 1 — B

Virus-Associated Trichodysplasia Spinulosa
The findings are highly distinctive for a unique folliculocentric viral syndrome seen in the setting of immunosuppression, mainly in the context of patients who are organ transplant recipients and/or on regimens of immunosuppressive drug therapy. The histopathology is very typical for trichodysplasia spinulosa, which is thought to be caused by a polyomavirus. Specifically, it is the trichodysplasia spinulosa polyomavirus 8.

The main abnormality is regarding the organization of the hair follicles, whereby there is an unusual hyperproliferation of the inner root sheath demonstrating irregular eosinophilic trichohyalin granules compatible with intracellular viral particles and not surprisingly there are significant dystrophic changes in the hair shaft.


Case 2 — D

Myofibroma
The findings are those of a soft tissue neoplasm which falls under the appellation of a solitary myofibroma. The lesion appears to exhibit an intravascular localization. The cardinal hallmarks of this benign soft tissue neoplasm are well exemplified by this case and include a dichotomous composition characterized by smooth muscle myomatous nodules with interposed hemangiopericytomatous-like foci. Histologically these lesions invariably exhibit a very characteristic biphasic pattern with whorled areas of spindled cells showing hyalinization and chondroid-like alteration of the background matrix with more interposed cellular spindled hypervascular hemangiopericytomatous areas. Phenotypically the myomatous nodules are desmin negative, although they do express smooth muscle actin and calponin.


Case 3 — C

Aneurysmal/Angiomatoid Fibrous Histiocytoma
The findings are characteristic for a rare form of fibrous histiocytoma representing angiomatoid fibrous histiocytoma. This neoplasm is a low-grade mesenchymal tumor of uncertain differentiation typically occurring in children and young adults on the extremities. Its morphologic hallmarks include a multinodular proliferation of spindled to ovoid cells associated with pseudoangiomatoid spaces. Low-grade atypia can be seen and indeed this particular case does exhibit random atypia of the neoplastic cell populace somewhat analogous to atypical fibrous histiocytoma. The neoplastic cells show focal staining for desmin in at least 40% of cases. In this case, weak cytoplasmic immunoreactivity of the neoplastic cells for desmin was noted. These tumors can recur; the metastatic rate is less than 1%, although it has been documented.


Case 4 — A

Blastomycosis
The findings are diagnostic of blastomycosis. A fairly robust neutrophilic and granulomatous response in association with the fungal elements was identified. The fungal forms are spherical with a thick refractile cell wall. They are multinucleate and have a broad-based budding.

 

Dr Magro is the director of dermatopathology at Weill Cornell Medicine in New York, NY. For more information, please visit www.weillcornelldermpath.com.

Disclosure: The author reports no relevant financial relationships.

Case 1.
A 49-year-old woman with a past medical history of immunosuppression presented with a nodule on the right lower nasofacial sulcus. (Figures A-E).

    a. Drug-induced follicular dystrophy
    b. Virus-associated trichodysplasia spinulosa
    c. Keratosis pilaris
    d. Lichen spinulosis


Case 2.
A 30-year-old woman presented with a subcutaneous mass of the right thigh. (Figures A-F)

    a. Leiomyoma
    b. Inflammatory myofibroblastic tumor
    c. Nodular fasciitis
    d. Myofibroma


Case 3.
A 20-year-old man presented with a nodule on the thigh. (Figures A-F)

    a. Angiosracoma
    b. Kaposi sarcoma
    c. Aneurysmal/angiomatoid fibrous histiocytoma
    d. Hemangioma


Case 4.
A 30-year-old woman presented with a cough, night sweats, and numerous ulcerated nodules of the bilateral arms. (Figures A-F)

    a. Blastomycosis
    b. Cryptococcosis
    c. Leishmaniasis
    d. Histoplasmosis

 

 

QUIZ ANSWERS

Case 1 — B

Virus-Associated Trichodysplasia Spinulosa
The findings are highly distinctive for a unique folliculocentric viral syndrome seen in the setting of immunosuppression, mainly in the context of patients who are organ transplant recipients and/or on regimens of immunosuppressive drug therapy. The histopathology is very typical for trichodysplasia spinulosa, which is thought to be caused by a polyomavirus. Specifically, it is the trichodysplasia spinulosa polyomavirus 8.

The main abnormality is regarding the organization of the hair follicles, whereby there is an unusual hyperproliferation of the inner root sheath demonstrating irregular eosinophilic trichohyalin granules compatible with intracellular viral particles and not surprisingly there are significant dystrophic changes in the hair shaft.


Case 2 — D

Myofibroma
The findings are those of a soft tissue neoplasm which falls under the appellation of a solitary myofibroma. The lesion appears to exhibit an intravascular localization. The cardinal hallmarks of this benign soft tissue neoplasm are well exemplified by this case and include a dichotomous composition characterized by smooth muscle myomatous nodules with interposed hemangiopericytomatous-like foci. Histologically these lesions invariably exhibit a very characteristic biphasic pattern with whorled areas of spindled cells showing hyalinization and chondroid-like alteration of the background matrix with more interposed cellular spindled hypervascular hemangiopericytomatous areas. Phenotypically the myomatous nodules are desmin negative, although they do express smooth muscle actin and calponin.


Case 3 — C

Aneurysmal/Angiomatoid Fibrous Histiocytoma
The findings are characteristic for a rare form of fibrous histiocytoma representing angiomatoid fibrous histiocytoma. This neoplasm is a low-grade mesenchymal tumor of uncertain differentiation typically occurring in children and young adults on the extremities. Its morphologic hallmarks include a multinodular proliferation of spindled to ovoid cells associated with pseudoangiomatoid spaces. Low-grade atypia can be seen and indeed this particular case does exhibit random atypia of the neoplastic cell populace somewhat analogous to atypical fibrous histiocytoma. The neoplastic cells show focal staining for desmin in at least 40% of cases. In this case, weak cytoplasmic immunoreactivity of the neoplastic cells for desmin was noted. These tumors can recur; the metastatic rate is less than 1%, although it has been documented.


Case 4 — A

Blastomycosis
The findings are diagnostic of blastomycosis. A fairly robust neutrophilic and granulomatous response in association with the fungal elements was identified. The fungal forms are spherical with a thick refractile cell wall. They are multinucleate and have a broad-based budding.

 

Dr Magro is the director of dermatopathology at Weill Cornell Medicine in New York, NY. For more information, please visit www.weillcornelldermpath.com.

Disclosure: The author reports no relevant financial relationships.

 

QUIZ ANSWERS

Case 1 — B

Virus-Associated Trichodysplasia Spinulosa
The findings are highly distinctive for a unique folliculocentric viral syndrome seen in the setting of immunosuppression, mainly in the context of patients who are organ transplant recipients and/or on regimens of immunosuppressive drug therapy. The histopathology is very typical for trichodysplasia spinulosa, which is thought to be caused by a polyomavirus. Specifically, it is the trichodysplasia spinulosa polyomavirus 8.

The main abnormality is regarding the organization of the hair follicles, whereby there is an unusual hyperproliferation of the inner root sheath demonstrating irregular eosinophilic trichohyalin granules compatible with intracellular viral particles and not surprisingly there are significant dystrophic changes in the hair shaft.


Case 2 — D

Myofibroma
The findings are those of a soft tissue neoplasm which falls under the appellation of a solitary myofibroma. The lesion appears to exhibit an intravascular localization. The cardinal hallmarks of this benign soft tissue neoplasm are well exemplified by this case and include a dichotomous composition characterized by smooth muscle myomatous nodules with interposed hemangiopericytomatous-like foci. Histologically these lesions invariably exhibit a very characteristic biphasic pattern with whorled areas of spindled cells showing hyalinization and chondroid-like alteration of the background matrix with more interposed cellular spindled hypervascular hemangiopericytomatous areas. Phenotypically the myomatous nodules are desmin negative, although they do express smooth muscle actin and calponin.


Case 3 — C

Aneurysmal/Angiomatoid Fibrous Histiocytoma
The findings are characteristic for a rare form of fibrous histiocytoma representing angiomatoid fibrous histiocytoma. This neoplasm is a low-grade mesenchymal tumor of uncertain differentiation typically occurring in children and young adults on the extremities. Its morphologic hallmarks include a multinodular proliferation of spindled to ovoid cells associated with pseudoangiomatoid spaces. Low-grade atypia can be seen and indeed this particular case does exhibit random atypia of the neoplastic cell populace somewhat analogous to atypical fibrous histiocytoma. The neoplastic cells show focal staining for desmin in at least 40% of cases. In this case, weak cytoplasmic immunoreactivity of the neoplastic cells for desmin was noted. These tumors can recur; the metastatic rate is less than 1%, although it has been documented.


Case 4 — A

Blastomycosis
The findings are diagnostic of blastomycosis. A fairly robust neutrophilic and granulomatous response in association with the fungal elements was identified. The fungal forms are spherical with a thick refractile cell wall. They are multinucleate and have a broad-based budding.

 

Dr Magro is the director of dermatopathology at Weill Cornell Medicine in New York, NY. For more information, please visit www.weillcornelldermpath.com.

Disclosure: The author reports no relevant financial relationships.