A Woman with a Pleural Effusion

Posted by • June 16th, 2016

2016-06-14_13-03-27

A 52-year-old woman presented with a unilateral pleural effusion. Several weeks later, uterine bleeding, pelvic fullness, and bloating developed. Magnetic resonance imaging revealed a large pelvic mass. Diagnostic procedures were performed. A new Case Record of the Massachusetts General Hospital summarizes.

Clinical Pearl

• What is the most common benign solid ovarian tumor?

Although only 4% of all ovarian masses are ovarian fibromas, they are the most common benign solid ovarian tumor.

Clinical Pearl

• What is the ovarian cancer symptom index?

A pelvic mass in a postmenopausal woman always raises concerns about ovarian cancer. The ovarian cancer symptom index is a tool used to predict or screen for ovarian cancer. According to this index, additional diagnostic testing for possible ovarian cancer is indicated in the presence of at least one of the following symptoms: abdominal pain; urinary frequency, urinary urgency, or both; and increased abdominal size, bloating, early satiety, or a combination thereof. The symptoms must be frequent and have occurred for less than a year. This index is 90% specific among women older than 50 years of age and 79.5% sensitive for advanced-stage disease.

Morning Report Questions

Q: Ovarian fibromas are associated with what syndromes?

A: Large fibromas (>10 cm in greatest diameter) are associated with ascites in 10 to 15% of cases and associated with ascites and a pleural effusion (the Demons–Meigs syndrome) in 1% of cases. In rare cases, fibromas may be part of the nevoid basal-cell carcinoma syndrome (Gorlin’s syndrome), which is characterized by the presence of multiple basal-cell carcinomas, keratocysts of the jaws, tumors of the central nervous system, fibromas, and skeletal malformations; in patients with this syndrome, ovarian fibromas are typically bilateral, multinodular, and associated with calcifications.

Figure 2. Imaging Studies of the Pelvis.

Figure 3. Resection Specimen.

Q: What are some of the features of the Demons–Meigs syndrome?

A: In 1887, Demons first described a syndrome in which various benign ovarian diseases were associated with the development of a pleural effusion. The description of this syndrome was further refined in a report of seven cases of ovarian fibroma with pleural effusion (usually unilateral and often on the right side) and pelvic fluid or ascites. Only 1 to 2% of patients with ovarian fibromas present with the Demons–Meigs syndrome. In such patients, the CA-125 level is often elevated, and thus it can be difficult to distinguish the Demons–Meigs syndrome from cancer. The hallmark of this syndrome is that removal of the ovarian mass results in permanent resolution of the pleural effusion.

19 Responses to “A Woman with a Pleural Effusion”

  1. Diego says:

    Excellent!

  2. Patrick Albert laizer says:

    Yes is true about the ovarian fibroma

  3. Anne Giles says:

    Since when are granulosa cell tumors benign? This is news to me, my oncologist, my gynecologic oncologist and the many women out there who have had to endure this cancer, the treatments, the follow-up, and for many the recurrences. Just because it is rare, slow-growing and not enough research has been done on it doesn’t make it benign. Check your facts before publishing something that may keep someone’s doctor from taking this disease seriously.

  4. Linda Langdale says:

    I am shocked to read in the above article that Granulosa Cell Tumours are listed as benign. They are most definitely malignant and I feel the opening statement to this article should be amended to correct this misleading inclusion.
    Very few doctors have any knowledge of Granulosa Cell Tumour and all too often get follow ups and treatments completely wrong leading to recurrences involving bowel, liver, spleen and lungs. I personally have just undergone my third major debulking surgery which required resection of my diaphragm and peritoneum. I had multiple tumours all over my abdomen, bladder, liver and in the retroperitoneal areas. I still have a tumour in my left lung.
    Please update your article to remove GCT from this “benign” list.
    Thank you!

  5. Rachael Milburn says:

    I have been dealing with Granulosa Cell Tumor since 2012. I’ve never been told it was benign. Upon diagnosis my tumor was ruptured. I underwent a total hysterectomy and 6 rounds of chemo. Things went ok until 2015 where they found several new Granulosa Cell Tumors. I had surgery to remove one but they couldn’t Pierre to get the other one. It was sandwiched in between my rectum and vagina. I went through chemo again to no avail. It caused my tumor to grow and put me in the hospital for two weeks. I’m currently doing rapid arc radiation, 33 treatments. My tumor has shrunk by half. This is only a small portion of the pain and torture I’ve endured for the last 4 years. So as you can see my Granulosa Cell Tumor is certainly no where near benign.

  6. Susan Rodgers says:

    Dear Sir/Madam:

    I have just read your article “A Woman with Pleural Effusion”. I am surprised and concerned you have stated that Granulosa Cell Tumor is a benign condition.

    This is false and misleading information to both the medical community and the public. GCT is a type of sex cord stromal tumor accounting for 2-5% of all ovarian cancers.

    I was diagnosed with GCT in 2013 after a laparoscopic procedure to remove a growth on my right ovary. Six weeks later I underwent a staging surgery at Roswell Park in Buffalo, NY that included a total hysterectomy and omentectomy.
    I continue to follow up every 6 mos with RPCI Gyn Clinic, which includes blood work for Inhibin B levels and pelvic exam.

    This CANCER is notorious for being slow growing with potential reoccurrence years after original diagnosis and requires long term follow up.

  7. Renee Bryant says:

    This article includes a huge error. Granulosa cell tumors of the ovary are malignant tumors. Publishing this kind of misinformation is one of the leading causes of women being under treated for a disease with a potentially fatal outcome.

    Please review this article and consider removing granulosa cell tumor of the ovary as a benign tumor. I am dying from this disease, I know first hand it is NOT benign and you are doing a terrible disservice to the women who will read this, doctors who will read this, and then believe that this disease does not have malignant potential.

  8. Ien fransen says:

    If Granulose cel is benigne…..then cure me,please!
    Must be no trouble for you.
    If gct is coming back evry time after operation and spread in the body and there is no cure for…..how come you call it benigne?
    On what ground do you label?
    Please answer my comment

  9. Lisa Miller says:

    I would be interested in knowing where you received your data that Granulosa Cell Tumors are benign. I was diagnosed in Dec. 2010 with a granulosa cell tumor by the University of Michigan. I was informed it was a cancer and because it had spread I was given 3 rounds of BEP chemotherapy. There is a cohort of women on Facebook that are GCT survivors that have seen multiple oncologists that would disagree that GCT is benign.

  10. Valene Houdyshell says:

    I am very surprised that you categorize granulosa-cell tumor as benign. If you ask the many women who suffer, and sometimes die from this malignant cancer they would tell you otherwise. Also, MD Anderson as well as most of the cancer research centers categorize it as malignant cancer. The women fighting this disease sometimes have to correct misinformed doctors that it is, in fact a malignant disease and were surprised to see your incorrect statement in this blog.. The many women who suffer with this malignant cancer would appreciate it if you would correct your misrepresentation of granulosa-cell tumor as benign. Thank you.

  11. Anne Caron says:

    Granulosa cell tumors are NOT benign. Please correct this incredible erroneous information which will be contributing to the deaths of many more women with GCT. Because of this misinformation to the gynecological medical community more operations will be done without the extreme caution that ovarian cancer tumor removals require. IF a ovarian cyst with a GCT is removed intact and without any spillage –i.e. 1a– there is a very good chance of escaping recurrence. Sadly, your misinformation makes this even less likely in future. With recurrence, the probably of dying early from increasingly serious and difficult to remove GCT metastases rises very steeply. Even at the 1a stage there is a significant chance of late recurrence, which is why knowledgeable sources stress that regular and lifelong follow-up with tests for Inhibin A, B, and/or AMH, as well as scans, is essential.

  12. Donna Sabens says:

    Dear Blog Writer, Please fact check your statement that GCT is a benign tumor. My oncologist, fellow GCT sisters, and even Aflac all know otherwise. A publication as prestigious as the New England Journal of Medicine must be held to a higher standard. Thank you.

  13. David Silon says:

    “Most ovarian masses are benign, including cystic lesions, endometriomas, mature teratomas, ovarian fibromas, fibrothecomas, and granulosa-cell tumors.”

    Respectfully, Granulosa Cell Tumors are not benign ovarian masses. They are a rare form of hormonally-active ovarian cancer (2-5% of all ovarian tumors) that is malignant in nature and tragically lethal in many cases. Ovarian GCT is an intractable, recurrent disease with few good treatments that tends to have a long natural history owing to occult microcellular seeding from tumor spillage/hematogenous transfer. However, GCT can also be very aggressive, where advanced cases usually include extensive metastatic spread throughout the abdomen and pelvis, including multiple hepatic and/or pulmonary metastases, among others. Bony and lymphatic metastases are uncommon. GCT’s primary biomarkers are Inhibin B, Inhibin A and MIS/AMH and these tumors have multiple biomarker phenotypes. CA125 is not a reliable biomarker for GCT, is often negative and is a possible source of a false negative interpretation in cases where an undiagnosed GCT is present.

    As a rare hormonally-active cancer, small, primary GCTs may completely disrupt menses, resulting in infertility and/or menorrhagia (with concomitant risk for endometrial adenocarcinoma based on the tumor’s estrogenic profile). These tumors require careful differential diagnosis (such as inhibin testing as an important diagnostic input for the majority of cases) vs. other benign gynecologic conditions. Further, in young girls, GCT is often diagnosed because of the onset of precocious puberty.

    During reproductive years, women with occult GCTs often experience difficulty with menses and pregnancy. Post-menopausally, primary GCTs are typically larger in size and women may present with ovarian torsion and critical abdominal symptoms, possibly with rupture/hemorrhage of the primary mass and hemoperitoneum. Spillage in GCT is usually of potent metastatic potential.

    Ovarian GCT deserves consideration and awareness at all levels of front-line gynecologic care and is certainly a worthy candidate for consideration when women present with symptoms that reflect chronic HPO-axis disruption and/or other hormonal anomalies, as above. While it’s never possible to catch every instance of disease at an early stage, especially in the case of rare disease, inhibin screening may be warranted when women present with apparent idiopathic HPO-axis disruption and TVU clearly shows the presence of ovarian cysts. Inhibin screening is certainly useful in monitoring recurrent disease, along with MIS/AMH.

    When considering whether an ovarian cyst might be benign or malignant, the only absolute confirmation is through surgical evaluation. And, while it may be true that most ovarian masses are benign, that assessment should not result in reduced diagnostic focus or very careful surgery for those who present with ovarian cysts of an unknown nature. Women who present with all forms of as-yet-undiagnosed ovarian cancer, rare or otherwise, deserve the utmost care and concern, as well as adept diagnostic synthesis to help ensure optimal care at initial presentation and a chance for the best long-term prognosis.

    Thanks for reading and for giving consideration to all those with Ovarian GCT.

    David Silon
    Founder/CTO
    StopGCT, Inc. Ovarian Cancer Research Foundation
    http://facebook.com/stopgct

  14. Betty Kradel says:

    Some forms of Theca Granulosa cell tumors are benign and others have low malignant potential. It is not a correct blanket statement that granulosa cell tumors (GCT) are benign. I believe this article does a great disservice to those in the medical community, who trust the NEJM for factual information and data, studying for a cure for GCT ovarian cancer. There are currently people in the United States who have hepatic metastasis and pulmonary metastasis of granulosa cell tumors who were originally told by practicing physicians that their cancer was benign. Please remove your statement of GCT being benign. I believe GCT being blanket categorized as ‘benign’, is a great disservice to GCT Research Foundation, Oncologists and GCT cancer patients fighting this disease.

  15. Dianne Baughman says:

    I am a member of a group of almost 500 worldwide who have been diagnosed with GCT. It IS malignant and I believe you are discounting GCT malignancy by your article stating it’s benign. Please – set the record straight or you may be finding yourselves on the wrong side of a lawsuit in the future.

  16. Terri Colclough says:

    I’m currently packing in order to relocate to Houston, Texas to receive daily radiation treatments for 6-7 weeks as a last ditch effort to treat my third recurrence of Granulosa Cell Tumor since 2012. I’ve undergone four surgeries, had intense chemotherapy that almost killed me, participated in a clinical trial composed of Avastin infusions and daily Anastrozole, and also took Letrozole for a six month period. NONE of the treatments have worked. Radiation is my last shot. It won’t cure me, but hopefully it will buy me more time. PLEASE print a retraction to your statement that Granulosa Cell Tumor is benign. Not only it is MALIGNANT, there are no treatments that appear to work for it! You have done all of us fighting this awful disease a terrible mis-service to print this erroneous information as even medical professionals know so little about GCT. Any physician or medical school student who reads this article and hasn’t had personal experience with women fighting this cancer will be MISINFORMED.

  17. Vivienne MacCarthy says:

    I do not have a medical background but would ask you please to connect with the Granulosa Cell Tumour Research Foundation http://www.gctf.org.nz/ to confirm your understanding of GCT’s.

  18. Sheila says:

    Why are you publishing inaccurate information? How can you state Granulosa cell tumors are benign? Where ar you getting your information? The facts and truth are, GCT is Cancer. GCT can recur. GCT needs more research. I suggest you redact your article and only publish information that is true

  19. Cynthia says:

    granulosa-cell tumors….benign?

    More research is needed on granulosa-cell tumors, I’ve been dealing with this for 12 years since diagnosed (2004) and undiagnosed for 22-23 years since symptoms appeared in 1993/94. Platinum-based chemo and 4 surgeries …it sure doesn’t feel benign….