Journal of Obstetrics and Gynaecology, 2015; Early Online: 1–2 © 2015 Informa UK, Ltd. ISSN 0144-3615 print/ISSN 1364-6893 online
CASE REPORT
Phyllodes tumour of vulva with prominent pseudoangiomatous stromal hyperplasia features: A case report and review of literature
Case report
1Department of Pathology & Laboratory Medicine, UT Health Science Center at Houston, Houston, TX, USA, and 2Department of Pathology, LBJ General Hospital, Houston, TX, USA
A 19-year-old female presented with a painful small lump on her genital area for 6 months. The lump became irritated and swollen during intercourse. The patient was not on any contraceptive or hormonal medicine. Her medical history was significant for irregular and heavy menstruation. On physical examination, a 1-cm lesion was noted between left labia minora and majora. The lesion was without drainage and non-tender to palpation. The clinical impression was sebaceous cyst. Surgical excision was performed.
DOI: 10.3109/01443615.2015.1007341
Pathological findings
B. Assylbekova1, P. Yan1,2 & M. Yang1,2
J Obstet Gynaecol Downloaded from informahealthcare.com by Kainan University on 04/25/15 For personal use only.
with prominent pseudoangiomatous stromal hyperplasia (PASH) features.
Correspondence: Mary Yang, MD, Department of Pathology, LBJ General Hospital, 5656 Kelley Street, 3PA 90.001A-C, Houston, TX 77026, USA. Tel: ⫹ 713-566-5264. Fax: ⫹ 713-566-5299. E-mail: [email protected]
Introduction Lesions arising from anogenital mammary-like glands (AGMLG) are rare and could be benign or malignant, and epithelial or stromal (Kazakov et al. 2010). Fibroepithelial lesions of AGMLG, including fibroadenoma and phyllodes tumour, are morphologically similar to their breast counterparts, but with much lower incidence. Phyllodes tumour of vulva is extremely rare with only thirteen reported cases in the English literature. We report a case of phyllodes tumour of vulva
The specimen consisted of a 0.8 ⫻ 0.3 ⫻ 0.3 cm rubbery, tan pink, fleshy portion of soft tissue. Microscopically, the tumour showed a typical leaf-like pattern of growth with bilayer epithelium. The stromal component showed prominent PASH features characterised by slit-like spaces and bland-looking spindle cells (Figure 1A and B). Nuclear atypia, mitosis, haemorrhage and necrosis were not seen. Immunohistochemical stains were performed for oestrogen receptor (ER), progesterone receptor (PR), pan-cytokeratin, musclespecific actin (MSA), p63, gross cystic disease fluid protein (GCDFP), CD31, CD34 and smooth muscle actin (SMA). The bilayer epithelial cells were positive for pan-cytokeratin. The luminal epithelial cells showed diffuse positivity to ER (Figure 1C) and PR, and patchy positivity to GCDFP. The basal layer was positive for MSA and p63. The stromal spindle cells were positive for CD34 (Figure 1D) and SMA, and negative for CD31, ER and PR. The results are consistent with
Figure 1. (A) Tumour showing typical leaf-like pattern of growth (haematoxylin and eosin [H&E], ⫻ 20). (B) Stroma showing PASH features characterised by slit-like spaces and bland-looking spindle cells (H&E, ⫻ 20). (C). Immunostaining for ER showing diffuse nuclear staining in the luminal layer of the epithelium and negative staining in the stromal spindle cells (ER, ⫻ 20). (D) Immunostaining for CD34 showing positive staining in the stromal spindle cells (CD34, ⫻ 20).
2
B. Assylbekova et al.
benign phyllodes tumour with prominent PASH features arising from AGMLG.
J Obstet Gynaecol Downloaded from informahealthcare.com by Kainan University on 04/25/15 For personal use only.
Discussion AGMLG in the vulva was first described by Hartung in 1872 (Kazakov et al. 2010), and has generally been considered to be caudal remnants of the milk ridges and ectopic breast tissue. However, van der Putte argued that ACMLG were normal constituents of anogenital area and that human mammary ridges develop only in the axillary-pectoral areas and never reach anogenital area as in other cetaceous animals (van der Putte 1994). Although histogenesis of ACMLG is still under debate, lesions arising from AGMLG strikingly resemble their breast counterparts. Fibroepithelial lesions arising from AGMLG are microscopically identical to their breast counterparts. About forty cases of fibroepithelial lesions, including thirteen cases of phyllodes tumour, arising from AGMLG in vulva were described in the literature (Fu et al. 2011; Giger et al. 2007; Heffermann et al. 2010; Kazakov et al. 2010; Mannan et al. 2010; Ozbudak et al. 2013). As in the breast, distinction between fibroadenoma and phyllodes tumour of AGMLG may be difficult. Fibroadenoma is often well circumscribed, and without hypercellular stroma. However, phyllodes tumour has irregular outlines, hypercellular stroma with periglandular condensation and typically leaf-like pattern of growth. The size of reported phyllodes tumours of vulva and the age of patients ranged from 0.3 to 12 cm and from 17 to 69 years, respectively. The tumour developed most commonly in unilateral labia minora, rarely in bilateral labia minora, periclitoral area and interlabial sulcus. Phyllodes tumour of the breast is categorised into benign, low grade and high grade, according to the degree of stromal atypia and mitotic rate. With the close morphological similarity of phyllodes tumour of AGMLG and breast, a similar approach was applied to phyllodes tumour of AGMLG. Majority of the reported cases of phyllodes tumour of vulva were benign, except one malignant (Fu et al. 2011), two low-grade (Kazakov et al. 2010) and one borderline tumours (Giger et al. 2007). However, due to the paucity of phyllodes tumour of vulva, further study and long term follow-up will be required to clarify the clinical implication and behavioural predictability of this categorisation. PASH in breast is a well-described entity with unclear pathogenesis. The lesional spindle cells are positive for CD34 and SMA, and negative for CD31, which are consistent with myofibroblastic phenotype. The current hypothesis of its pathogenesis is exaggerated
response of mammary myofibroblasts to endogenous or exogenous hormonal stimuli, particularly progesterone (Virk and Khan 2010). PASH was first described in AGMLG in 2005 by Kazakov et al. To the best of our knowledge, by far, only five cases of phyllodes tumour of vulva with focal PASH features have been reported in the English literature (Kazakov et al. 2010). In conclusion, we report a rare case of phyllodes tumour of vulva with prominent PASH features. Although definitive ER and PR receptors were not demonstrable immunohistochemically in the stromal spindle cells, a possible hormonal aetiology was suggested by the patient’s history of irregular menstruation. Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.
References Fu L, Lau S, Roy I, Ferenczy A. 2011. Phyllodes tumor with malignant stromal morphology of the vulva: a case report and review of literature. International Journal of Gynecologic Pathology 30:198–202. Giger OT, Lacoste E, Honegger C et al. 2007. Expression of the breast differentiation antigen NY-BR-1 in a phyllodes tumor of the vulva. Virchows Archives 450:471–474. Heffermann TP, Sarose VR, Hoffman B et al. 2010. Recurrent phyllodes tumor of the vulva: A case report with review of diagnostic criteria and differential diagnosis. International Journal of Gynecological Pathology 29:294–297. Kazakov DV, Bisceglia M, Mukensnabl P et al. 2005. Pseudoangiomatous stromal hyperplasia in lesions involving anogenital mammary like glands. American Journal of Surgical Pathology 29:1243–1246. Kazakov DV, Spagnolo DV, Kacerovska D et al. 2011. Lesions of anogenital mammary-like glands: an update. Advances in Anatomic Pathology 18:1–28. Kazakov DV, Spagnolo DV, Stewart CJ et al. 2010. Fibroadenoma and phyllodes tumors of anogenital mammary-like glands: A series of 13 neoplasms in 12 cases, including mammry type juvenile fibroadenoma, fibroadenoma with lacation changes, and neurofibromatosis-associated pseudoangiomatous stromal hyperplasia with multinucleated giant cells. American Journal of Surgical Pathology 34:95–103. Mannan AASR, Kahvic M, Abdel Aziz AH. 2010. Phyllodes tumor of the vulva: report of a rare case and review of the literature. American Journal of Dermatopathology 32:384–386. Ozbudak IH, Akkaya H, Akkaya B et al. 2013. Phyllodes tumor of the vulva: report of two cases. Turk Patoloji Dergisi 29:73–76. Van der Putte SC. 1994. Mammary-like glands of the vulva and their disorders. International Journal of Gynecologic Pathology 13:150–160. Virk RK, Khan A. 2010. Pseudoangiomatous stromal hyperplasia: an overview. Archives of Pathology and Laboratory Medicine 134:1070–1074.
CASE REPORT
Phyllodes tumour of vulva with prominent pseudoangiomatous stromal hyperplasia features: A case report and review of literature
Case report
1Department of Pathology & Laboratory Medicine, UT Health Science Center at Houston, Houston, TX, USA, and 2Department of Pathology, LBJ General Hospital, Houston, TX, USA
A 19-year-old female presented with a painful small lump on her genital area for 6 months. The lump became irritated and swollen during intercourse. The patient was not on any contraceptive or hormonal medicine. Her medical history was significant for irregular and heavy menstruation. On physical examination, a 1-cm lesion was noted between left labia minora and majora. The lesion was without drainage and non-tender to palpation. The clinical impression was sebaceous cyst. Surgical excision was performed.
DOI: 10.3109/01443615.2015.1007341
Pathological findings
B. Assylbekova1, P. Yan1,2 & M. Yang1,2
J Obstet Gynaecol Downloaded from informahealthcare.com by Kainan University on 04/25/15 For personal use only.
with prominent pseudoangiomatous stromal hyperplasia (PASH) features.
Correspondence: Mary Yang, MD, Department of Pathology, LBJ General Hospital, 5656 Kelley Street, 3PA 90.001A-C, Houston, TX 77026, USA. Tel: ⫹ 713-566-5264. Fax: ⫹ 713-566-5299. E-mail: [email protected]
Introduction Lesions arising from anogenital mammary-like glands (AGMLG) are rare and could be benign or malignant, and epithelial or stromal (Kazakov et al. 2010). Fibroepithelial lesions of AGMLG, including fibroadenoma and phyllodes tumour, are morphologically similar to their breast counterparts, but with much lower incidence. Phyllodes tumour of vulva is extremely rare with only thirteen reported cases in the English literature. We report a case of phyllodes tumour of vulva
The specimen consisted of a 0.8 ⫻ 0.3 ⫻ 0.3 cm rubbery, tan pink, fleshy portion of soft tissue. Microscopically, the tumour showed a typical leaf-like pattern of growth with bilayer epithelium. The stromal component showed prominent PASH features characterised by slit-like spaces and bland-looking spindle cells (Figure 1A and B). Nuclear atypia, mitosis, haemorrhage and necrosis were not seen. Immunohistochemical stains were performed for oestrogen receptor (ER), progesterone receptor (PR), pan-cytokeratin, musclespecific actin (MSA), p63, gross cystic disease fluid protein (GCDFP), CD31, CD34 and smooth muscle actin (SMA). The bilayer epithelial cells were positive for pan-cytokeratin. The luminal epithelial cells showed diffuse positivity to ER (Figure 1C) and PR, and patchy positivity to GCDFP. The basal layer was positive for MSA and p63. The stromal spindle cells were positive for CD34 (Figure 1D) and SMA, and negative for CD31, ER and PR. The results are consistent with
Figure 1. (A) Tumour showing typical leaf-like pattern of growth (haematoxylin and eosin [H&E], ⫻ 20). (B) Stroma showing PASH features characterised by slit-like spaces and bland-looking spindle cells (H&E, ⫻ 20). (C). Immunostaining for ER showing diffuse nuclear staining in the luminal layer of the epithelium and negative staining in the stromal spindle cells (ER, ⫻ 20). (D) Immunostaining for CD34 showing positive staining in the stromal spindle cells (CD34, ⫻ 20).
2
B. Assylbekova et al.
benign phyllodes tumour with prominent PASH features arising from AGMLG.
J Obstet Gynaecol Downloaded from informahealthcare.com by Kainan University on 04/25/15 For personal use only.
Discussion AGMLG in the vulva was first described by Hartung in 1872 (Kazakov et al. 2010), and has generally been considered to be caudal remnants of the milk ridges and ectopic breast tissue. However, van der Putte argued that ACMLG were normal constituents of anogenital area and that human mammary ridges develop only in the axillary-pectoral areas and never reach anogenital area as in other cetaceous animals (van der Putte 1994). Although histogenesis of ACMLG is still under debate, lesions arising from AGMLG strikingly resemble their breast counterparts. Fibroepithelial lesions arising from AGMLG are microscopically identical to their breast counterparts. About forty cases of fibroepithelial lesions, including thirteen cases of phyllodes tumour, arising from AGMLG in vulva were described in the literature (Fu et al. 2011; Giger et al. 2007; Heffermann et al. 2010; Kazakov et al. 2010; Mannan et al. 2010; Ozbudak et al. 2013). As in the breast, distinction between fibroadenoma and phyllodes tumour of AGMLG may be difficult. Fibroadenoma is often well circumscribed, and without hypercellular stroma. However, phyllodes tumour has irregular outlines, hypercellular stroma with periglandular condensation and typically leaf-like pattern of growth. The size of reported phyllodes tumours of vulva and the age of patients ranged from 0.3 to 12 cm and from 17 to 69 years, respectively. The tumour developed most commonly in unilateral labia minora, rarely in bilateral labia minora, periclitoral area and interlabial sulcus. Phyllodes tumour of the breast is categorised into benign, low grade and high grade, according to the degree of stromal atypia and mitotic rate. With the close morphological similarity of phyllodes tumour of AGMLG and breast, a similar approach was applied to phyllodes tumour of AGMLG. Majority of the reported cases of phyllodes tumour of vulva were benign, except one malignant (Fu et al. 2011), two low-grade (Kazakov et al. 2010) and one borderline tumours (Giger et al. 2007). However, due to the paucity of phyllodes tumour of vulva, further study and long term follow-up will be required to clarify the clinical implication and behavioural predictability of this categorisation. PASH in breast is a well-described entity with unclear pathogenesis. The lesional spindle cells are positive for CD34 and SMA, and negative for CD31, which are consistent with myofibroblastic phenotype. The current hypothesis of its pathogenesis is exaggerated
response of mammary myofibroblasts to endogenous or exogenous hormonal stimuli, particularly progesterone (Virk and Khan 2010). PASH was first described in AGMLG in 2005 by Kazakov et al. To the best of our knowledge, by far, only five cases of phyllodes tumour of vulva with focal PASH features have been reported in the English literature (Kazakov et al. 2010). In conclusion, we report a rare case of phyllodes tumour of vulva with prominent PASH features. Although definitive ER and PR receptors were not demonstrable immunohistochemically in the stromal spindle cells, a possible hormonal aetiology was suggested by the patient’s history of irregular menstruation. Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.
References Fu L, Lau S, Roy I, Ferenczy A. 2011. Phyllodes tumor with malignant stromal morphology of the vulva: a case report and review of literature. International Journal of Gynecologic Pathology 30:198–202. Giger OT, Lacoste E, Honegger C et al. 2007. Expression of the breast differentiation antigen NY-BR-1 in a phyllodes tumor of the vulva. Virchows Archives 450:471–474. Heffermann TP, Sarose VR, Hoffman B et al. 2010. Recurrent phyllodes tumor of the vulva: A case report with review of diagnostic criteria and differential diagnosis. International Journal of Gynecological Pathology 29:294–297. Kazakov DV, Bisceglia M, Mukensnabl P et al. 2005. Pseudoangiomatous stromal hyperplasia in lesions involving anogenital mammary like glands. American Journal of Surgical Pathology 29:1243–1246. Kazakov DV, Spagnolo DV, Kacerovska D et al. 2011. Lesions of anogenital mammary-like glands: an update. Advances in Anatomic Pathology 18:1–28. Kazakov DV, Spagnolo DV, Stewart CJ et al. 2010. Fibroadenoma and phyllodes tumors of anogenital mammary-like glands: A series of 13 neoplasms in 12 cases, including mammry type juvenile fibroadenoma, fibroadenoma with lacation changes, and neurofibromatosis-associated pseudoangiomatous stromal hyperplasia with multinucleated giant cells. American Journal of Surgical Pathology 34:95–103. Mannan AASR, Kahvic M, Abdel Aziz AH. 2010. Phyllodes tumor of the vulva: report of a rare case and review of the literature. American Journal of Dermatopathology 32:384–386. Ozbudak IH, Akkaya H, Akkaya B et al. 2013. Phyllodes tumor of the vulva: report of two cases. Turk Patoloji Dergisi 29:73–76. Van der Putte SC. 1994. Mammary-like glands of the vulva and their disorders. International Journal of Gynecologic Pathology 13:150–160. Virk RK, Khan A. 2010. Pseudoangiomatous stromal hyperplasia: an overview. Archives of Pathology and Laboratory Medicine 134:1070–1074.
