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Rapid Growth of Dermatofibrosarcoma Protuberans Associated with Bilateral Adrenalectomy for Cushing's Syndrome.

Furudate S, Fujimura T, Hashimoto A, Aiba S - Case Rep Dermatol (2011)

Bottom Line: We describe a 50-year-old Japanese patient with dermatofibrosarcoma protuberans (DFSP) rapidly growing after bilateral adrenalectomy for Cushing's syndrome that reduced the serum level of cortisol from 17.1 to 0.8 mg/dl.Therefore, the hypersecretion of glucocorticoids in Cushing's syndrome might suppress the development of DFSP.To the best of our knowledge, this is the first case of rapid growth of DFSP that may be associated with bilateral adrenalectomy for Cushing's syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.

ABSTRACT
We describe a 50-year-old Japanese patient with dermatofibrosarcoma protuberans (DFSP) rapidly growing after bilateral adrenalectomy for Cushing's syndrome that reduced the serum level of cortisol from 17.1 to 0.8 mg/dl. It is known that glucocorticoids decrease the transcriptions of the COL1A1 gene and the PDGFB gene, which is under the direct control of the COL1A1 gene in most DFSP. Therefore, the hypersecretion of glucocorticoids in Cushing's syndrome might suppress the development of DFSP. To the best of our knowledge, this is the first case of rapid growth of DFSP that may be associated with bilateral adrenalectomy for Cushing's syndrome.

No MeSH data available.


Related in: MedlinePlus

Densely packed, monomorphic, plump, spindle cells arranged in a storiform pattern in the central area of the tumor nodule, and, in the peripheral lesion, spindle cells diffusely infiltrating around the tumor (a, b). Immunohistochemical staining for CD34 (c) and Factor XIIIa (d). a Original magnification ×100; b–d original magnification ×400.
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Figure 2: Densely packed, monomorphic, plump, spindle cells arranged in a storiform pattern in the central area of the tumor nodule, and, in the peripheral lesion, spindle cells diffusely infiltrating around the tumor (a, b). Immunohistochemical staining for CD34 (c) and Factor XIIIa (d). a Original magnification ×100; b–d original magnification ×400.

Mentions: A 50-year-old Japanese woman visited our outpatient clinic with a 20-year history of an asymptomatic, about 3 mm in diameter, subcutaneous nodule on her breast. One year before, this nodule rapidly enlarged after she underwent bilateral adrenalectomy for Cushing's syndrome. On her initial visit, physical examination revealed a brown-colored, elastic hard, well-demarcated nodule on the left side of her breast (fig. 1). The tumor mass was 20 × 18 mm in size. An excisional biopsy showed densely packed, monomorphic, plump, spindle cells arranged in a storiform pattern in the central area of the tumor nodule and spindle cells infiltrating into the subcutis of the peripheral lesion (fig. 2a, b). The tumor cells were positive for CD34 and negative for factor XIIIa (fig. 2c, d), SMA, and S100 (data not shown). Based on the above-mentioned findings, we diagnosed this patient as having DFSP. Interestingly, the serum levels of cortisol before and after bilateral adrenalectomy were 17.1 and 0.8 mg/dl, respectively. We excised the tumor with a 3-cm surgical margin of mammary gland tissue. A half year after surgical treatment, there was no evidence of local tumor recurrence.


Rapid Growth of Dermatofibrosarcoma Protuberans Associated with Bilateral Adrenalectomy for Cushing's Syndrome.

Furudate S, Fujimura T, Hashimoto A, Aiba S - Case Rep Dermatol (2011)

Densely packed, monomorphic, plump, spindle cells arranged in a storiform pattern in the central area of the tumor nodule, and, in the peripheral lesion, spindle cells diffusely infiltrating around the tumor (a, b). Immunohistochemical staining for CD34 (c) and Factor XIIIa (d). a Original magnification ×100; b–d original magnification ×400.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3104867&req=5

Figure 2: Densely packed, monomorphic, plump, spindle cells arranged in a storiform pattern in the central area of the tumor nodule, and, in the peripheral lesion, spindle cells diffusely infiltrating around the tumor (a, b). Immunohistochemical staining for CD34 (c) and Factor XIIIa (d). a Original magnification ×100; b–d original magnification ×400.
Mentions: A 50-year-old Japanese woman visited our outpatient clinic with a 20-year history of an asymptomatic, about 3 mm in diameter, subcutaneous nodule on her breast. One year before, this nodule rapidly enlarged after she underwent bilateral adrenalectomy for Cushing's syndrome. On her initial visit, physical examination revealed a brown-colored, elastic hard, well-demarcated nodule on the left side of her breast (fig. 1). The tumor mass was 20 × 18 mm in size. An excisional biopsy showed densely packed, monomorphic, plump, spindle cells arranged in a storiform pattern in the central area of the tumor nodule and spindle cells infiltrating into the subcutis of the peripheral lesion (fig. 2a, b). The tumor cells were positive for CD34 and negative for factor XIIIa (fig. 2c, d), SMA, and S100 (data not shown). Based on the above-mentioned findings, we diagnosed this patient as having DFSP. Interestingly, the serum levels of cortisol before and after bilateral adrenalectomy were 17.1 and 0.8 mg/dl, respectively. We excised the tumor with a 3-cm surgical margin of mammary gland tissue. A half year after surgical treatment, there was no evidence of local tumor recurrence.

Bottom Line: We describe a 50-year-old Japanese patient with dermatofibrosarcoma protuberans (DFSP) rapidly growing after bilateral adrenalectomy for Cushing's syndrome that reduced the serum level of cortisol from 17.1 to 0.8 mg/dl.Therefore, the hypersecretion of glucocorticoids in Cushing's syndrome might suppress the development of DFSP.To the best of our knowledge, this is the first case of rapid growth of DFSP that may be associated with bilateral adrenalectomy for Cushing's syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.

ABSTRACT
We describe a 50-year-old Japanese patient with dermatofibrosarcoma protuberans (DFSP) rapidly growing after bilateral adrenalectomy for Cushing's syndrome that reduced the serum level of cortisol from 17.1 to 0.8 mg/dl. It is known that glucocorticoids decrease the transcriptions of the COL1A1 gene and the PDGFB gene, which is under the direct control of the COL1A1 gene in most DFSP. Therefore, the hypersecretion of glucocorticoids in Cushing's syndrome might suppress the development of DFSP. To the best of our knowledge, this is the first case of rapid growth of DFSP that may be associated with bilateral adrenalectomy for Cushing's syndrome.

No MeSH data available.


Related in: MedlinePlus