11 November 2010 - Case #190
All cases are archived on our website. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. To subscribe or unsubscribe to Case of the Month or our other email lists, click here.
Thanks to Dr. Tim Bracey, Royal Cornwall Hospital (United Kingdom), for contributing this case.
Advertisement
Case #190
Clinical history:
A 60 year old woman with no previous history had an ellipse of skin submitted from her right cheek, with a clinical diagnosis of basal cell carcinoma.
Microscopic images:
What is your diagnosis?
Diagnosis: Storiform collagenoma
Discussion:
Storiform collagenoma, first described in 1991, is a well circumscribed, hypocellular lesion with focal, heavy collagen deposition in a distinctive interweaving, storiform pattern, with prominent elongated clefts between the collagen bundles (Am J Dermatopathol 1991;13:122). Multinucleated giant cells have been described (Am J Surg Pathol 1998;22:557). Solitary lesions are also called sclerosing fibroma.
Storiform collagenoma usually occurs in young and middle aged adults, and presents as a slow growing solitary nodule up to 1 cm on the head, neck or upper extremities. It may be solitary but multiple nodules are associated with Cowden disease, a rare, autosomal dominant disorder with multiple hamartomatous neoplasms of ectodermal, mesodermal and endodermal origin; an increased risk of breast, thyroid or endometrial carcinoma; and disorders of the CNS, musculoskeletal system, GI system and female genital tract (eMedicine: Cowden Disease (Multiple Hamartoma Syndrome) [Accessed 18 April 2024]). Rarely, storiform collagenoma may be the presenting feature of Cowden disease (J Clin Pathol 2007;60:840). Cowden disease is considered part of the spectrum of various disorders with mutations in the PTEN gene, which collectively are termed PTEN hamartoma tumor syndrome.
The nodular stells are often immunoreactive for CD34 and vimentin, with prominent reticulin fibers. Scattered dendritic cells are factor XIIIa positive. The nodules are negative for keratin, EMA, CEA and elastic stains.
The differential diagnosis includes dermatofibroma, which has infiltrative margins, pseudoepitheliomatous hyperplasia and foamy histiocytes and diffuse factor XIIIa staining. Sclerotic lipoma may appear similar but has a variable number of S100+ and fat stain+ adipocytes interspersed within the fibrosclerotic stroma (Histopathology 1997;31:174). Perineurioma is composed primarily of perineurial cells but may occasionally appear similar. It demonstrates variable CD34 staining but is also typically EMA positive and S100 negative (Am J Surg Pathol 2005;29:845).
These tumors have benign behavior and no treatment is necessary. Solitary tumors may be excised for cosmetic reasons.
All cases are archived on our website. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. To subscribe or unsubscribe to Case of the Month or our other email lists, click here.
Thanks to Dr. Tim Bracey, Royal Cornwall Hospital (United Kingdom), for contributing this case.
Introducing EP Clones:
Rabbit Monoclonal Antibodies
for Anatomic Pathology
As the leader of Rabbit Monoclonal Antibody (RabMAb) technology, Epitomics is pleased to introduce EP Clones, a new class of high quality antibodies for anatomic pathology. Using Epitomics patented technology, combining the high specificity of monoclonal antibodies with high affinity of rabbit antibodies, a group of RabMAbs were generated against popular and well-known pathology targets (e.g. Her2, ER, PR, Ki-67, CD Markers). Fully validated (ASR, IVD labeled), EP clones are ideal for FFPE tissues and shows superior specificity and sensitivity compared to traditional antibody options.
For more information contact us at 877-772-2622, IVDinfo@epitomics.com, or visit our website at
http://www.epitomics.com/diagnostics.
Website news:
(1) Support PathologyOutlines.com by doing your holiday shopping at Amazon.com. Click here, or on any Amazon banner on the left side of our Home Page to visit Amazon.com. By visiting Amazon via these links, they will send us 4-5% of the cost of all items you purchase (out of their profits), at no additional cost to you.
(2) We are currently updating topics one at a time in the Bladder, Cervix-Cytology and Coagulation chapters, and hope to have the chapters completed soon.
Visit and follow our Blog to see recent updates to the website.
(1) Support PathologyOutlines.com by doing your holiday shopping at Amazon.com. Click here, or on any Amazon banner on the left side of our Home Page to visit Amazon.com. By visiting Amazon via these links, they will send us 4-5% of the cost of all items you purchase (out of their profits), at no additional cost to you.
(2) We are currently updating topics one at a time in the Bladder, Cervix-Cytology and Coagulation chapters, and hope to have the chapters completed soon.
Visit and follow our Blog to see recent updates to the website.
Case #190
Clinical history:
A 60 year old woman with no previous history had an ellipse of skin submitted from her right cheek, with a clinical diagnosis of basal cell carcinoma.
Microscopic images:
CD34
What is your diagnosis?
Click here for diagnosis and discussion:
Diagnosis: Storiform collagenoma
Discussion:
Storiform collagenoma, first described in 1991, is a well circumscribed, hypocellular lesion with focal, heavy collagen deposition in a distinctive interweaving, storiform pattern, with prominent elongated clefts between the collagen bundles (Am J Dermatopathol 1991;13:122). Multinucleated giant cells have been described (Am J Surg Pathol 1998;22:557). Solitary lesions are also called sclerosing fibroma.
Storiform collagenoma usually occurs in young and middle aged adults, and presents as a slow growing solitary nodule up to 1 cm on the head, neck or upper extremities. It may be solitary but multiple nodules are associated with Cowden disease, a rare, autosomal dominant disorder with multiple hamartomatous neoplasms of ectodermal, mesodermal and endodermal origin; an increased risk of breast, thyroid or endometrial carcinoma; and disorders of the CNS, musculoskeletal system, GI system and female genital tract (eMedicine: Cowden Disease (Multiple Hamartoma Syndrome) [Accessed 18 April 2024]). Rarely, storiform collagenoma may be the presenting feature of Cowden disease (J Clin Pathol 2007;60:840). Cowden disease is considered part of the spectrum of various disorders with mutations in the PTEN gene, which collectively are termed PTEN hamartoma tumor syndrome.
The nodular stells are often immunoreactive for CD34 and vimentin, with prominent reticulin fibers. Scattered dendritic cells are factor XIIIa positive. The nodules are negative for keratin, EMA, CEA and elastic stains.
The differential diagnosis includes dermatofibroma, which has infiltrative margins, pseudoepitheliomatous hyperplasia and foamy histiocytes and diffuse factor XIIIa staining. Sclerotic lipoma may appear similar but has a variable number of S100+ and fat stain+ adipocytes interspersed within the fibrosclerotic stroma (Histopathology 1997;31:174). Perineurioma is composed primarily of perineurial cells but may occasionally appear similar. It demonstrates variable CD34 staining but is also typically EMA positive and S100 negative (Am J Surg Pathol 2005;29:845).
These tumors have benign behavior and no treatment is necessary. Solitary tumors may be excised for cosmetic reasons.
