Giant superficial angiomyxoma of the vulva: A case report and review of the literature

Hyun Soo Kim, Gou Young Kim, Sung Jig Lim, Kyung Do Ki, Hyun Cheol Kim

Research output: Contribution to journalReview article

9 Citations (Scopus)

Abstract

Superficial angiomyxomas (SAMs) are rare, benign cutaneous tumors frequently involving the subcutis. Only 15 cases of SAM involving the vulva have been reported, ranging from 0.9 to 4 cm in diameter. A 26-year-old woman presented with a 7-year history of a large, pedunculated cutaneous mass on the left labium major, measuring 12.5 × 11 × 10.5 cm and mimicking a soft tissue sarcoma. The mass was relatively well-circumscribed, but unencapsulated and multilobulated. Microscopically, the mass showed a conglomerate of moderately-to-sparsely cellular angiomyxoid lobules. Each lobule consisted of scattered spindle-shaped or stellate tumor cells set in an abundant myxoid stroma. Thin-walled, small-to-medium-sized blood vessels were distributed diffusely throughout the stroma. Scattered stromal neutrophils were also observed. No large vessels or plexiform capillaries were apparent. There was no perivascular accentuation of stromal cells or smooth muscle bundles. The tumor cells constantly expressed vimentin, CD34, CD44 and S-100, but none expressed estrogen receptors (ERs) and progesterone receptors (PRs), desmin or cytokeratin. Together, these findings were diagnostic of a SAM. Giant SAMs of the vulva can mimic aggressive angiomyxomas (AAMs) and angiomyofibroblastomas (AMB), as well as soft tissue sarcomas. Giant SAMs should be included in the differential diagnosis of vulvar soft tissue tumors.

Original languageEnglish
Pages (from-to)672-677
Number of pages6
JournalJournal of Cutaneous Pathology
Volume37
Issue number6
DOIs
Publication statusPublished - 2010 Jun 1

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Myxoma
Vulva
Sarcoma
Neoplasms
Skin
Desmin
Vimentin
Progesterone Receptors
Stromal Cells
Keratins
Estrogen Receptors
Smooth Muscle
Blood Vessels
Neutrophils
Differential Diagnosis

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

Cite this

Kim, Hyun Soo ; Kim, Gou Young ; Lim, Sung Jig ; Ki, Kyung Do ; Kim, Hyun Cheol. / Giant superficial angiomyxoma of the vulva : A case report and review of the literature. In: Journal of Cutaneous Pathology. 2010 ; Vol. 37, No. 6. pp. 672-677.
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abstract = "Superficial angiomyxomas (SAMs) are rare, benign cutaneous tumors frequently involving the subcutis. Only 15 cases of SAM involving the vulva have been reported, ranging from 0.9 to 4 cm in diameter. A 26-year-old woman presented with a 7-year history of a large, pedunculated cutaneous mass on the left labium major, measuring 12.5 × 11 × 10.5 cm and mimicking a soft tissue sarcoma. The mass was relatively well-circumscribed, but unencapsulated and multilobulated. Microscopically, the mass showed a conglomerate of moderately-to-sparsely cellular angiomyxoid lobules. Each lobule consisted of scattered spindle-shaped or stellate tumor cells set in an abundant myxoid stroma. Thin-walled, small-to-medium-sized blood vessels were distributed diffusely throughout the stroma. Scattered stromal neutrophils were also observed. No large vessels or plexiform capillaries were apparent. There was no perivascular accentuation of stromal cells or smooth muscle bundles. The tumor cells constantly expressed vimentin, CD34, CD44 and S-100, but none expressed estrogen receptors (ERs) and progesterone receptors (PRs), desmin or cytokeratin. Together, these findings were diagnostic of a SAM. Giant SAMs of the vulva can mimic aggressive angiomyxomas (AAMs) and angiomyofibroblastomas (AMB), as well as soft tissue sarcomas. Giant SAMs should be included in the differential diagnosis of vulvar soft tissue tumors.",
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Giant superficial angiomyxoma of the vulva : A case report and review of the literature. / Kim, Hyun Soo; Kim, Gou Young; Lim, Sung Jig; Ki, Kyung Do; Kim, Hyun Cheol.

In: Journal of Cutaneous Pathology, Vol. 37, No. 6, 01.06.2010, p. 672-677.

Research output: Contribution to journalReview article

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T1 - Giant superficial angiomyxoma of the vulva

T2 - A case report and review of the literature

AU - Kim, Hyun Soo

AU - Kim, Gou Young

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AU - Kim, Hyun Cheol

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N2 - Superficial angiomyxomas (SAMs) are rare, benign cutaneous tumors frequently involving the subcutis. Only 15 cases of SAM involving the vulva have been reported, ranging from 0.9 to 4 cm in diameter. A 26-year-old woman presented with a 7-year history of a large, pedunculated cutaneous mass on the left labium major, measuring 12.5 × 11 × 10.5 cm and mimicking a soft tissue sarcoma. The mass was relatively well-circumscribed, but unencapsulated and multilobulated. Microscopically, the mass showed a conglomerate of moderately-to-sparsely cellular angiomyxoid lobules. Each lobule consisted of scattered spindle-shaped or stellate tumor cells set in an abundant myxoid stroma. Thin-walled, small-to-medium-sized blood vessels were distributed diffusely throughout the stroma. Scattered stromal neutrophils were also observed. No large vessels or plexiform capillaries were apparent. There was no perivascular accentuation of stromal cells or smooth muscle bundles. The tumor cells constantly expressed vimentin, CD34, CD44 and S-100, but none expressed estrogen receptors (ERs) and progesterone receptors (PRs), desmin or cytokeratin. Together, these findings were diagnostic of a SAM. Giant SAMs of the vulva can mimic aggressive angiomyxomas (AAMs) and angiomyofibroblastomas (AMB), as well as soft tissue sarcomas. Giant SAMs should be included in the differential diagnosis of vulvar soft tissue tumors.

AB - Superficial angiomyxomas (SAMs) are rare, benign cutaneous tumors frequently involving the subcutis. Only 15 cases of SAM involving the vulva have been reported, ranging from 0.9 to 4 cm in diameter. A 26-year-old woman presented with a 7-year history of a large, pedunculated cutaneous mass on the left labium major, measuring 12.5 × 11 × 10.5 cm and mimicking a soft tissue sarcoma. The mass was relatively well-circumscribed, but unencapsulated and multilobulated. Microscopically, the mass showed a conglomerate of moderately-to-sparsely cellular angiomyxoid lobules. Each lobule consisted of scattered spindle-shaped or stellate tumor cells set in an abundant myxoid stroma. Thin-walled, small-to-medium-sized blood vessels were distributed diffusely throughout the stroma. Scattered stromal neutrophils were also observed. No large vessels or plexiform capillaries were apparent. There was no perivascular accentuation of stromal cells or smooth muscle bundles. The tumor cells constantly expressed vimentin, CD34, CD44 and S-100, but none expressed estrogen receptors (ERs) and progesterone receptors (PRs), desmin or cytokeratin. Together, these findings were diagnostic of a SAM. Giant SAMs of the vulva can mimic aggressive angiomyxomas (AAMs) and angiomyofibroblastomas (AMB), as well as soft tissue sarcomas. Giant SAMs should be included in the differential diagnosis of vulvar soft tissue tumors.

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