Tuesday, August 26, 2014

President's Message August 2014

Dear Friends and Colleagues,

I am delighted to inform you that, thanks to Grace Kim’s hard work and dedication, the Hans Popper Hepatopathology Society is now officially a Non-Profit Organization and has garnered 501(3)(c) tax-exempt status officially recognized by the IRS. Additionally, the HPHS bank account has been set up locally in California. Further details from Grace are found in this newsletter.

The HPHS Companion Society Meeting is scheduled for March 22, 2015 from 8:30am-12noon. The title is “Diagnostic Challenges and Update in Hepatopathology”. We have created a program that will not only be very educational to our members but will hopefully be interesting to non-members as well.

The Business meeting will immediately follow the companion meeting. Members are encouraged to attend as we will need to vote on some modifications to the bylaws as a result of the tax-exempt status of the HPHS, as well as some changes to the duties and responsibilities of the members of the Executive Committee, and finally to officially create a Website Committee. In the meantime, I would like members to volunteer to serve on this committee. Please email me directly (mariaisabel.fiel@mountsinai.org) if you are interested.  In the near future, we will have the ability to check off on-line our interest in committee membership when we pay our yearly dues. Currently, Arief Suriawinata is overseeing the website with the help of Vince Hoang, Grace Kim’s Administrative Assistant. Vince has replaced Julie Gutierrez, who was an invaluable help to Beth Brunt with the membership dues, correspondence, and all other HPHS matters for many years. We thank Julie for all of her efforts throughout the years!

The Call for Abstracts for the “Pathologist-in-Training Award” is now official. Deadline for submission to Dele Odeyi (oyedele.adeyi@uhn.ca), Chair of the Education Committee, is March 6, 2015. Please encourage your residents and fellows to submit their work to the HPHS.

Finally, please encourage both your junior and senior colleagues to officially become members of the HPHS. Residents and fellows can become Trainee members without having to pay any dues. They can easily transition to Regular membership once they finish their training. Application to membership is available on the website. Please email completed application to Maha Guindi (Maha.Guindi@cshs.org), chair of the membership committee.

I hope everyone is having a great summer. I look forward to seeing you in Boston.

M. Isabel Fiel
President,
Hans Popper Hepatopathology Society

Time to support the Hans Popper Hepatopathology Society - Membership dues for 2014-2015 or Donations

The Hans Popper Hepatopathology Society has an EIN (Federal Tax Identification Number for use to identify a business entity) and has become incorporated as:  Hans Popper Hepatopathology Society, Inc.  We are also tax-exempt (501(c)(3)); therefore your membership dues and donations are now tax-deductible. 

Membership categories:

Regular Member:  Any individual who has completed a postdoctoral program in Pathology or another specialty of Medicine, and has earned certification as a specialist in Pathology or another specialty of Medicine, with demonstrated interest and involvement in hepatobiliary pathology.  Dues: $50/year

Associate Member:   Any person holding a doctorate, or doctorate equivalent, degree, who is enrolled in a post-doctoral educational program in Pathology (PhD, post-doctoral fellowship, residency or fellowship) or another specialty of Medicine, and who has demonstrated interest in hepatobiliary pathology.  Dues:  None; limited to five years only.

Corresponding Member:  Any individual who satisfies the requirements for Regular Membership or Associate Membership, but does not reside in the United States or Canada.  No dues.  Corresponding members can apply for regular membership.

Ways you can contribute (membership dues or donations).

1.      Bank Transfer: If you have a Wells Fargo account, you can transfer funds using the following bank information:
a.    Routing #: 121042882
b.    Account #: 8558608389

2.      PayPal: You may now contribute via PalPay using your personal credit card.

Membership Dues - 2014


Donations:

3.     Bank Checks: You can send a check via U.S. mail:  Make checks payable to: Hans Popper Hepatopathology Society, Inc. Include “membership dues” or “general donation” in the memo line of your check. Send checks to:

Grace E. Kim, Secretary-Treasurer
Hans Popper Hepatopathology Society, Inc.
505   Parnassus Avenue M561 Box 0102
San Francisco, CA 94143-0102

When using option 1 and 3, you will receive an electronic receipt from the Hans Popper Hepatopathology Society, Inc. to file your tax deductions.


Monday, August 11, 2014

Interesting Case August 2014


Clinical history

The patient is an 81 year old male with no significant past medical history who presented with abdominal pain, nausea and weight loss of several months duration.

Labs

ALT 15 U/L, AST 55 U/L, ALK PHOS 81 U/L, total bilirubin 0.3 mg/dl. Tumor makers: AFP 1 IU/ml, CEA 2.4 ng/ml CA19-9 55 U/ml, PSA 11.35 ng/ml.

Radiology

CT scan showed four liver masses which measured up to 3.6 cm, a 6.9 cm left kidney mass and an 8.9 cm mesenteric mass. A CT-guided biopsy of the liver mass was performed.

Microscopic images


Figure 1
Figure 2
Figure 3
Figure 4
Figure 5, HMB-45
Figure 6, Melan A
Figure 7, CD10
Figure 8, AE1/AE3
Figure 9, S-100

Diagnosis

Metastatic malignant epithelioid angiomyolipoma.

Discussion

This tumor demonstrates a proliferation of predominantly large epithelioid cells with abundant eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli (Figure 1). Focal tumor cell spindling is seen (Figure 2). Scattered ganglion-like cells and multinucleated giant cells are present (Figure 3). Mitoses, including atypical mitotic figures, are present (Figure 3 and Figure 4). No necrosis is identified. The tumor cells are positive by immunohistochemistry (IHC) for HMB45 (Figure 5), Melan A (Figure 6), CD10 (Figure 7), MiTF, and CD68; they are negative for cytokeratin AE1/AE3 (Figure 8), S100 (Figure 9), CAM 5.2, CD117, CD34, SMA, desmin, calponin, myogenin, and inhibin.

The differential diagnosis includes, but is not limited to, renal cell carcinoma with sarcomatoid differentiation, adrenal cortical carcinoma, melanoma, malignant GIST, undifferentiated/sarcomatoid carcinoma and malignant epithelioid angiomyolipoma. Combined epithelioid and spindle cell morphology, large ganglion-like cells and multinucleated giant cells are characteristic of epithelioid angiomyolipoma (E-AML). Positive IHC for HMB45, Melan A, MiTF and CD68, together with negative IHC for epithelial markers and S100 help confirm the diagnosis.  However, as we are always taught, “melanoma is the great mimicker” and rarely melanoma can be S-100 negative.

The usual renal angiomyolipoma (AML) is considered a benign mesenchymal tumor composed of a variable portion of fat, smooth muscle cells, and abnormal thick-walled vessels. E-AML is a rare variant and potentially malignant neoplasm composed partially or entirely of epithelioid cells. Up to one-third of E-AML patients experience local recurrences and/or distant metastases. Features that favor malignant E-AML include nuclear atypical in ≥70% of the epithelioid cells, ≥2 mitotic figures per 10 high power fields, atypical mitotic figures, and necrosis; the presence of 3 or all of these features is predictive of aggressive behavior (Brimo F, Robinson B, Guo C et al. Am J Surg Pathol 2010;34:715-722). E-AMLs are immunoreactive for one or more melanocytic markers (e.g., HMB45, Melan A, and MiTF). They often express smooth muscle markers such as SMA and desmin. The absence of S100 staining can help distinguish E-AML from melanoma. The absence of staining for epithelial markers can help distinguish E-AML from carcinoma.

Patient follow up:
Shortly after the diagnosis of malignant E-AML was made in August of 2013, the patient was started on Afinitor 10 mg daily (also called everolimus, an established medication for AML). He had an excellent initial response (CT scan in early 2014 showed significantly reduced size of the mesenteric mass with stable liver and kidney masses). However, the treatment was held several times due to severe side effects including hyponatremia, diarrhea and fevers. Afinitor was re-started in July of 2014 at a very low dose. Currently, one year since diagnosis, the patient is doing reasonable well with no new masses.

Contributed by
Wendy Cao, MD