I have PASH and need an expert who can help me.?

Question

I was diagnosed with 6 cm PASH,and was operated on at major, high profile medical center last October. They did not remove all of it and it is growing and painful. I need a doctor who can help me. I can go anywhere.

Answer 1

Background

This is a rare but benign tumor of the breast. Its importance lies in the diagnostic skills of the pathologist, distinguishing it from a malignancy. PSH stands for pseudoangiomatous stromal hyperplasia. It is a diangosis that only a pathologist can render by examining the breast tissue under the microscope.

OUTLINE


Disease Associations
Pathogenesis
Laboratory/Radiologic/Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/
Immunohistochemistry/
Electron Microscopy
Differential Diagnosis
Prognosis and Treatment
Commonly Used Terms
Internet Links


DISEASE ASSOCIATIONS CHARACTERIZATION
FIBROADENOMA

Pseudoangiomatous hyperplasia of mammary stroma: a case of pure type after removal of fibroadenoma.

Fukunaga M.

Department of Pathology, The Jikei University School of Medicine, Tokyo, Japan.
APMIS 2001 Feb;109(2):113-6 Abstract quote
A case of pure pseudoangiomatous hyperplasia of the mammary stroma after removal of a fibroadenoma is described. The lesion, which was found in the right breast of a 40-year-old woman, was a well-circumscribed non-encapsulated, rubbery, lobulated mass measuring 40x40x35 mm.

Histologically, it consisted predominantly of a proliferation of spindle cells with interanastomosing vascular-like arrangements in the interlobular or interductal stroma. Neither cytological atypia nor mitotic figures were observed. The stroma contained abundant collagen with focal hyalinization. Focally, epithelial elements showed mild ductal hyperplasia. There was no fibroadenoma. Immunohistochemically, the spindle cells were positive for vimentin, CD34, alpha-smooth muscle actin, muscle actin, CD34, calponin, and progesterone receptors.

Ultrastructurally, many spindle cells had thin elongated cytoplasmic processes, which enveloped pseudovascular spaces containing a few collagenous fibrils, indicating fibroblastic cells. This lesion should be distinguished from other mammary fibrous and vascular lesions with which it may be confused.

GYNECOMASTIA
Rapid growth of pseudoangiomatous hyperplasia of mammary stroma in axillary gynecomastia in an immunosuppressed patient.

Seidman JD, Borkowski A, Aisner SC, Sun CC.

Department of Pathology, University of Maryland Medical System, University of Maryland Hospital, Baltimore.
Arch Pathol Lab Med 1993 Jul;117(7):736-8 Abstract quote
Pseudoangiomatous hyperplasia of mammary stroma is a rare, benign mesenchymal proliferation that sometimes mimics angiosarcoma.

To our knowledge, it has not been reported in men. We report the first case of this unusual entity in a man. This case was most unusual in that it occurred in a clinical setting of immunosuppression, in axillary gynecomastia, and was growing rapidly.



Gynecomastia in type-1 neurofibromatosis with features of pseudoangiomatous stromal hyperplasia with giant cells. Report of two cases.

Damiani S, Eusebi V.

Department of Oncology, Marcello Malpighi of the University of Bologna, Italy.
Virchows Arch 2001 May;438(5):513-6 Abstract quote
We describe the histological finding in two cases of gynecomastia in patients with von Recklinghausen's disease. The histological and immunohistochemical features of the two cases were reviewed and compared with those of five cases of gynecomastia in men without clinical evidence of neurofibromatosis.

In both patients bearing von Recklinghausen's disease, the breast stroma showed features consistent with pseudoangiomatous stromal hyperplasia (PASH). It was characterised by anastomosing empty spaces lined by spindle and multinucleated giant cells which were positive with CD34 and anti-vimentin antisera and negative with anti-FVIII and CD31 antisera. In two of five of the control cases without neurofibromatosis, the mammary stroma showed focal areas with features of PASH, but no multinucleated giant cells were present in any case. PASH with giant cells should be recognised as a feature of gynecomastia in von Recklinghausen's disease.

The presence of multinucleated giant cells is very unusual and, although more cases have to be studied, these cells seem to be a feature of PASH occurring in patients with von Recklinghausen's disease.

HIV
Pseudoangiomatous hyperplasia of mammary stroma in an HIV patient.

de Saint Aubain Somerhausen N, Larsimont D, Cluydts N, Heymans O, Verhest A.

Department of Pathology, Institut Jules Bordet, Bruxelles, Belgium.
Gen Diagn Pathol 1997 Dec;143(4):251-4 Abstract quote
Pseudoangiomatous hyperplasia of the mammary stroma is a benign mesenchymal proliferative lesion. It is rarely observed as a tumor mass that has to be differentiated from angiosarcoma.

We give a review of the literature and report a case unusual in that it occurred in a clinical setting of HIV infection, was rapidly growing, and has been the largest described so far.




PATHOGENESIS CHARACTERIZATION
MYOFIBROBLASTS

Pseudoangiomatous stromal hyperplasia (PASH). A mammary stromal tumor with myofibroblastic differentiation.

Powell CM, Cranor ML, Rosen PP.

Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021.
Am J Surg Pathol 1995 Mar;19(3):270-7 Abstract quote
Pseudoangiomatous stromal hyperplasia (PASH) is frequently a microscopic incidental finding in breast biopsies performed for benign or malignant disease. However, it may also produce a mass lesion.

We reviewed PASH seen first as a tumor in 40 women aged 14 to 67 years (mean, 37 years). All but one lesion were clinically palpable. The exceptional tumor was found by mammography. The mass, typically unilateral, was usually diagnosed clinically as a fibroadenoma. Most specimens contained a well-circumscribed tumor with a firm white-gray cut surface. In six cases, there was no discrete gross lesion in the surgical specimen. Microscopically, there was a spectrum of pathological stromal changes ranging from classical PASH with anastomosing slit-shaped spaces outlined by flat, bland spindle cells to more proliferative lesions composed of bundles of plump spindle cells that obscured the underlying pseudoangiomatous architecture in the most florid lesions.

The spindle cells were vimentin and CD34 positive and factor VIII negative. In more cellular fascicular lesions, the stromal cells acquired desmin and actin positivity.These immunohistochemical features were consistent with myofibroblastic histogenesis of PASH. Reactivity for progesterone receptor (PR) typically exceeded estrogen receptor (ER) in the nuclei of stromal and glandular cells. In most lesions, the nuclei of stromal spindle cells were ER negative. The majority of the patients were treated by excisional biopsy. One lesion, incompletely excised, spontaneously regressed. One patient had bilateral mastectomies. Follow-up was 0.6-11 years (mean, 4.5 years). Five patients had ipsilateral recurrences, and two had subsequent contralateral PASH. The morphological spectrum of cellular proliferation and staining qualities indicates that the myofibroblast plays a major role in the histogenesis of PASH.

The pathogenesis of PASH remains uncertain, but aberrant reactivity of myofibroblasts to endogenous or exogenous hormones is likely to be an important factor. Simple excision is adequate treatment initially and for infrequent recurrences, Diffuse PASH occasionally presents a difficult management problem that may necessitate mastectomy.




LABORATORY/
RADIOLOGIC/
OTHER TESTS
CHARACTERIZATION
RADIOLOGIC

Pseudoangiomatous stromal hyperplasia: mammographic, sonographic, and clinical patterns.

Cohen MA, Morris EA, Rosen PP, Dershaw DD, Liberman L, Abramson AF.

Department of Radiology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Radiology 1996 Jan;198(1):117-20 Abstract quote
PURPOSE: To analyze the mammographic, ultrasonographic (US), and clinical patterns of pseudoangiomatous stromal hyperplasia (PASH).

MATERIALS AND METHODS: Preoperative mammographic and US images, clinical records, and histologic assessments were reviewed in seven cases of PASH as the sole component or dominant stromal component of a clinically or mammographically defined mass lesion. In five, a PASH tumor was diagnosed at image-guided 14-gauge core-needle biopsy; in two, it was diagnosed at surgical excisional biopsy.

RESULTS: Four women were premenopausal, two were postmenopausal, and one 40-year-old patient had iatrogenic primary amenorrhea. Four of seven masses were palpable; three were discovered at mammography. Four masses enlarged over 6 months to 5 years. At mammography, all masses lacked calcifications; six were well defined, and one was spiculated. One was inapparent at US; the remaining six were well defined and hypoechoic.

CONCLUSION: The diagnosis of this rare lesion with image-guided breast biopsy or surgical biopsy is consistent with a well-defined uncalcified mass at mammography and a solid hypoechoic mass at US, usually in a premenopausal woman.


Pseudoangiomatous stromal hyperplasia: mammographic and sonographic appearances.

Polger MR, Denison CM, Lester S, Meyer JE.

Department of Radiology, Brigham and Women's Hospital, Boston, MA 02115, USA.
AJR Am J Roentgenol 1996 Feb;166(2):349-52 Abstract quote
OBJECTIVE: Pseudoangiomatous stromal hyperplasia is a benign, localized form of stromal overgrowth with a probable hormonal etiology. Our purpose is to describe the mammographic, sonographic, and clinical features of this entity.

MATERIALS AND METHODS: A retrospective review of breast imaging studies and demographic information for seven patients identified as having pseudoangiomatous stromal hyperplasia was performed. The patients were chosen from a 1-year period during which 1661 breast biopsies were performed.

RESULTS: The patients were 36-61 years old. Six were premenopausal, and the one postmenopausal woman was on hormone replacement therapy. Three patients wee evaluated palpable breast lumps, and four were asymptomatic. All seven women had noncalcified masses that measured 1.1-11 cm and that were visible by mammography. The border characteristics were as follows: three masses were well circumscribed, two were partly circumscribed, and two were indistinct, probably because they were obscured by overlying parenchyma. Sonography was performed for five patients; lesions were visible in four. All four of these lesions were solid and circumscribed. Five of the masses had increased in size since earlier studies, and one palpable mass was found by physical examination to have grown over time. Two patients had a local recurrence of pseudoangiomatous stromal hyperplasia. Surgical excision was performed for three patients, large-core biopsy was performed for three patients, and both surgery and large-core biopsy were performed for one patient.

ONCLUSION: Pseudoangiomatous stromal hyperplasia should be included in the differential diagnosis of a circumscribed or partially circumscribed mass, especially in the premenopausal population. These masses often grow over time and can recur locally. Pathologic diagnosis of the lesion may be difficult unless the pathologist is aware of the presence of a mass lesion and appreciates the stromal changes characteristic of such a lesion.

Imaging appearances of pseudoangiomatous hyperplasia of mammary stroma.

Kirkpatrick UJ, Burrows C, Loughran CF.

Department of Surgery, Macclesfield District General Hospital, Cheshire SK10 3BL, UK.
Clin Radiol 2000 Jul;55(7):576-8
LABORATORY MARKERS



GROSS APPEARANCE/
CLINICAL VARIANTS CHARACTERIZATION
GENERAL
Pseudoangiomatous stromal hyperplasia of the breast.

Castro CY, Whitman GJ, Sahin AA.

Department of Pathology, The University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA.
Am J Clin Oncol 2002 Apr;25(2):213-6 Abstract quote
Pseudoangiomatous stromal hyperplasia is a benign proliferative lesion of the mammary stroma that rarely presents as a localized mass. We describe the clinical, radiologic, cytologic, and histologic features of a case of pseudoangiomatous stromal hyperplasia that presented as a rapidly growing breast lesion in a 36-year-old woman.


VARIANTS
ANOGENITAL
Pseudoangiomatous Stromal Hyperplasia in Lesions Involving Anogenital Mammary-Like Glands.

Kazakov DV, Bisceglia M, Mukensnabl P, Michal M.

From *Sikl's Department of Pathology, Charles University, Medical Faculty Hospital, Pilsen, Czech Republic; and daggerDepartment of Pathology, IRCCS-Ospedale "Casa Sollievo della Sofferenza," San Giovanni Rotondo, Italy.

Am J Surg Pathol. 2005 Sep;29(9):1243-1246. Abstract quote

Pseudoangiomatous stromal hyperplasia (PASH), first reported in 1986, is nowadays a well-recognized change in the breast.

We present three cases of lesions involving anogenital mammary-like glands demonstrating this feature. All patients were females (ages, 42, 43, and 53 years). Each presented with a solitary, 1.5- to 2-cm asymptomatic nodule. Locations included the perianal area, perineum, and labium majus.

Histopathologically, one lesion was classified as low-grade phyllodes tumor, another as fibroadenoma, and in the remaining case PASH was found in the background of mild hyperplasia of anogenital mammary-like glands and substantial lipomatous metaplasia. In all lesions, PASH had an identical appearance to that in the breast, that is open, slit-like, often anastomosing channels devoid of erythrocytes and lined by discontinuous, often attenuated, inconspicuous cells without atypia or mitotic activity set in a hyalinized collagenous stroma. Quantitatively, PASH ranged in the above cases, forming a relatively small focus in the fibroadenoma and being quite extensive in the remaining two cases. In the phyllodes tumor, PASH areas exhibited focal hypercellularity and presence of myoid cells. In none of the cases were there cells with intranuclear inclusions or multinucleated cells. The lesions were surgically excised. Two patients with follow-up were disease-free at one and three years after the operation.

As to our knowledge, PASH has not been previously described in the anogenital area, this feature seems to have been either overlooked or is genuinely rare in this location. It may occur in a preexisting lesion of anogenital mammary-like glands or may apparently by itself produce a clinically detectable lesion. The clinicopathologic features of PASH in the anogenital area seem to be identical to those in the breast.
MALE BREAST

Pseudoangiomatous hyperplasia of male breast.

Badve S, Sloane JP.

Department of Histopathology, Royal Marsden Hospital, Sutton, Surrey, UK.
Histopathology 1995 May;26(5):463-6 Abstract quote
Ninety-three male breast specimens have been examined for the presence of pseudoangiomatous hyperplasia of the mammary stroma which has hitherto been described almost exclusively in females.

Forty-four cases (47.4%) showed some degree of hyperplasia, varying from small microscopic foci to extensive change involving 90% of the mammary tissue. All but one were found in association with gynaecomastia, early and intermediate stage.

The association between pseudoangiomatous hyperplasia and benign proliferative lesions mirrors that reported in the female breast, and our findings suggest that the change may represent a stage in the maturation of newly formed mammary stroma.

MASS

Pseudoangiomatous stromal hyperplasia: presentation as a mass in the female nipple.

Iancu D, Nochomovitz LE.

Department of Pathology, Winthrop-University Hospital, Mineola, New York 11501, USA.
Breast J 2001 Jul-Aug;7(4):263-5 Abstract quote
Pseudoangiomatous stromal hyperplasia (PASH) is a benign, localized fibroblastic and myofibroblastic overgrowth that occurs almost exclusively in premenopausal women as a painless, palpable intramammary mass.

The lesion has a pale, fibrous, and homogeneous cut surface, is typically well circumscribed, and may have a diameter of 2.0-15 cm. Its ramifying slits lined by flattened myofibroblastic cells are apt to be mistaken for vascular spaces, leading to an erroneous diagnosis of angiosarcoma. The etiology of the condition is unknown, but a relationship to myofibroblastoma has been postulated. Hormonal factors, too, are thought to play a developmental role.

The potential for PASH to create a palpable breast mass has been only quite recently advanced in the medical literature, and it has evidently not been reported in the nipple.

Answer 2

Pash Tumor

Answer 3

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Answer 5

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Answer 8

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