What is LPD?

"...the enigmatic disorder known as disseminated peritoneal leiomyomatosis. DPL is very rare condition in which "fibroid" tumorlets numbering from a handful to hundreds are scattered about the peritoneal surfaces and omentum in the abdominal cavity. The way that we think about DPL changed after [Quade] laboratory published about paper showing that DPL tumorlets are "metastatic" from a single tumor clone. Despite this uniclonal origin, in most cases, DPL behaves as a clinically benign process, with many cases presenting incidentally and the rest of cases presenting because of symptoms related to the mass effect of these many tumorlets. In a very small number of cases, DPL seems to "transform" into leiomyosarcoma, another uniclonal, but malignant smooth muscle tumor. Consequently, for most patients, chemotherapy has little effect and the side effects cause more harm than benefit. DPL, like uterine fibroids (benign smooth muscle tumors that we diagnose as leiomyomas), is composed of benign smooth muscle tumor cells that are hormonally sensitive, and reducing the level of estrogen and progesterone by GnRH agonists (e.g., Lupron) and oopherectomy seems to help, but these obviously leave the patient in a postmenopausal state, which has important implications for bone, heart, and reproductive health. A few cases of hormone-secreting ovarian tumors have been associated with DPL as well. If mass effect symptoms are present, debulking surgery helps as long as the procedure is through and that the microscopic tumorlets don't grow back.

...patients with DPL should see a gynecologic oncologist (a doctor trained in the surgical management of tumors of the female genital tract), preferably one who practice in a university setting as they will be more likely to have managed a case or two. Unfortunately, there is no one out there that has taken care of large numbers of DPL patients. In New York, I would consider going to the Memorial-Sloane Kettering Cancer Center. Here at [Brigham and Women’s Hospital ], I would recommend seeing Dr. Mike Muto or Dr. Ross Berkowitz (or another member of their group)."



"Leiomyomatosis peritonealis disseminata (LPD), also known as diffuse peritoneal leiomyomatosis, is a rare disease in which multiple smooth muscle or smooth muscle-like nodules develop subperitoneally in any part of the abdominal cavity [1].

These nodules, though histologically benign, cannot be distinguished macroscopically from peritoneal carcinomatosis.
The aetiology is thought to be smooth muscle metaplasia of the subperitoneal mesenchyme [2]. About 100 documented cases were found in the English language literature; LPD patients are mainly females of reproductive age [3], and only rarely have cases affecting men been reported [4,5].

The condition is associated with high levels of exogenous and endogenous female gonadal steroids (e.g. pregnancy, prolonged exposure to oral contraceptives and/or combined hormonal replacement therapy, granulomatous cell tumours of the ovary) [4,6], indicating that oestrogens and progestins play an important role in the pathogenesis of LPD as they do in leiomyomata uteri.

Most LPD cases are clinically benign, and in some instances the lesions may partially or completely regress [5,7]. Alternatively, LPD may progress, recur or (rarely) undergo malignant transformation [8].

LPD is most common in women of reproductive age. More than half of all patients are pregnant or taking oral contraceptives at the time of diagnosis [14].

Quade et al. showed (in 1997) that LPD has molecular-genetic and cytogenetic features suggesting that individual tumourlets are monoclonal, with a pathogenesis similar to leiomyomata uteri. In LPD, the smooth muscle cells are influenced by oestrogens [7,15], and sex steroid receptors have been identified in nearly all cases [16]. LPD can be associated with other oestrogen-dependent diseases including endometriosis, ovarian clear cell carcinoma, endometrial carcinoma [17] and ovarian fibrothecoma [2]. Recently, two cases of development of LPD and ovarian Brenner tumour during tamoxifen [cancer drug] therapy have been reported [18-22].

Although LPD is most common in premenopausal women, cases have been reported in postmenopausal women using [23] or not using [13,24,25] hormone replacement therapy (HRT). The identification of luteinizing hormone (LH) receptors in LPD nodules from a postmenopausal woman suggests that the typical postmenopausal increase in LH levels might affect the pathogenesis of this condition [22].

Recently, familial occurrence of LPD has been described, showing an autosomal dominant model with varying degrees of penetrance [26].

Most patients with LPD present without specific symptoms and many documented cases of LPD have been discovered incidentally during surgery (caesarean section, laparotomy or laparoscopy). Sometimes patients may present with mostly non-specific symptoms, such as irregular, heavy uterine bleeding and pain or a mass in the lower abdomen [27], discomfort, urinary frequency (due to the effect of the mass on the bladder), gastrointestinal bleeding and peritonitis (following erosion of LPD implants in the bowel wall) [7,13,18,28]. Sometimes patients experience symptoms directly related to LPD: urosepsis secondary to obstruction of the ureters, and an acute abdomen due to ovarian torsion [29].

Sonographic and CT findings reported in the literature include non-specific, solid, and complex soft tissue masses that are often large and mimic a leiomyomatous uterus. In some cases, the masses grow in a fashion similar to that of normal uterine parenchyma, whereas others demonstrate heterogeneous enhancement. Diagnosis may be confused with peritoneal carcinomatosis if the masses are present diffusely throughout the abdomen and pelvis. Peritoneal carcinomatosis, however, is often associated with tumour cake, ascites and liver metastases, which have not been reported with LPD [29].

Magnetic Resonance (MR) findings include masses similar in signal intensity to skeletal muscle or uterine parenchyma, and when sarcomatous transformation occurs these are not significantly different from the features of benign implants. If the masses are located in the pelvis adjacent to the iliac vessels, they may be confused with lymphadenopathy [5,14,30-33]. Moreover, some multiple, pedunculated leiomyomas arising from the uterus may mimic LPD implants. Final diagnosis relies on histological examination [34] and immunohistochemical evaluation.

Sometimes, LPD may recur in patients taking HRT [13,24,25] even after hysterectomy and bilateral salpingo-oophorectomy [23], or in patients who have undergone in vitro fertilization [35]. Matthews and Speers reported a patient who died after a fourth recurrence of LPD and distant metastases 10 years after hysterectomy. Each recurrence seems to be more likely to produce morphological evidence of sarcoma [23].

Malignant transformation of LPD is uncommon and only ten cases have been documented in the English literature [8,29]. Of these, only three occurred in postmenopausal women (so seven cases of malignant transformation in premenopausal women)[29,36]. The interval between initial detection of LPD and the development of sarcoma varies from synchronous diagnosis to 8 years.

In most reports of malignant LPD, no history of oestrogen exposure was found. Bekkers hypothesized that LPD without exogenous or increased endogenous oestrogen exposure, and without expression of ER/PR by tumour cells, may represent a different entity carrying a higher risk of malignant transformation [3].

On the basis of these observations, we can affirm that no established guidelines exist regarding the management of LPD. However, therapy needs to be individualised according to the patient's age, hormonal and reproductive status and symptomatology. Different drugs (gonadotropin realising hormone agonist (GnRH agonist), megestrol acetate, danazol) have been considered in some cases but with poor results. If intestinal and bladder mass effect symptoms are prominent, a surgical approach is indicated [37]."

BMC Cancer. 2006; 6: 127.
Published online 2006 May 10. doi: 10.1186/1471-2407-6-127.
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1481579
Showing posts with label fibroids. Show all posts
Showing posts with label fibroids. Show all posts

Thursday, September 20, 2007

Sloan-Kettering Sucks

I contacted Sloan-Kettering and they are not interested in this case because they do not classify it as cancer. They would be happy to see me for a 3000 USD consultation, though that would be with a general surgeon, not an oncologist. ?!?!

Thursday, September 13, 2007

LPD after hormonal ablation

There are cases of LPD occuring after hysterectomy, after oophorectomy. To my mind that would indicate that hormonal ablation, while logically the first line of defense, is not the cure and the cause needs to be looked into more carefully. Sorry to those women. Who will look into the cause?

Saturday, August 25, 2007

Hope?

I just bumped into this and it gives me hope that somebody is, or was, looking into LPD.

http://www.brighamandwomens.org/WRHRprogram/FacultyResearchSummaries.aspx

REPRODUCTIVE CANCER UNIT
Unit Chief: Daniel Cramer, M.D.,Department of Obstetrics, Gynecology and Reproductive BiologyBrigham and Women's Hospital,

Quade Laboratory
The first major focus of the Quade laboratory is the pathobiology of mesenchymal neoplasms arising in the female genital tract. These tumors range from extremely common leiomyomata to rare, yet often fatal leiomyosarcomas and unusual, quasi-malignant proliferations. Our goal is to identify the molecular factors that cause these tumors and determine their malignant potential. Previously, we have shown that disseminated peritoneal leiomyomatosis, a clinically benign condition in which hundreds of fibroid-like tumorlets stud the peritoneum and omentum, is in fact a clonal metastatic process. ...

Monday, August 6, 2007

Juicing?

LPD is sometimes thought of as cancer and sometimes like a uterine fibroid. Here is something that suggests cancer and fribroids should not be treated the same. So what should someone with LPD do? Follow the cancer theory or the fibroid theory?

"http://www.healingcancernaturally.com/gerson1.html

Q. Can fibroid tumors be dissolved in the same manner?
A. Fibroid tumors are mostly benign. Benign tumors take 10 to 20 times as much time to absorb as malignant tumors. This goes for adhesions and scars. Fibroid and benign tumors are dissolved only very slowly because they are not abnormal. It is difficult for the parenteral system to bring its digestive powers to bear on these benign tumors. But when they turn malignant, then they are quickly dissolved."

Thursday, July 26, 2007

Frankensense Oil?

Here is something I bumped into:

http://www.namiscc.org/Recovery/2003/Spring/OilsForRecovery.htm

Cancerous growths and tumors, including fibroids, are far too common with antidepressant use. I believe it is a good idea to use frankincense oil as a protection against that possibility after being on antidepressants. I have seen massive amounts of hard lumps under the arms disappear within weeks using frankensense oil. ...

Herbs For Fibrous Masses

An excerpt from an article titled, "Abdominal Adhesions: Prevention and Treatment" located at
http://www.itmonline.org/arts/adhesions.htm

...The herbs from the table above are ingredients in traditional and modern formulas used in resolving problems that are relevant to fibrous masses and adhesions.

For example, a traditional formula for treating pain due to old trauma, which may reflect existence of adhesions, is Sanleng Heshang Tang (12). It is comprised of 12 herbs for regulating circulation of qi and blood and alleviating pain; the formula includes sparganium, zedoaria, myrrh, frankincense, and tang-kuei.

A formula for "movable or immovable mass in the abdomen," Huoluo Xiaoling Dan, is made with just four herbs: salvia, myrrh, frankincense, and tang-kuei.

A modern formula developed for treating uterine fibroids, Gong Zheng Tang, includes sparganium, zedoaria, achyranthes, tang-kuei, and persica (13). ...

Tuesday, July 24, 2007

LPD Is Cancer

When I asked the Office of Rare Diseases about LPD, they sent me an article about cancer. Here are some excerpts. The entire article is titled Peritoneal Cancer and there is a link in the section called articles.

"...Other described primary peritoneal cancers and tumors include ... leiomyomatosis peritonealis disseminata (LPD)...Most cases of leiomyomatosis peritonealis disseminata have been discovered in reproductive-aged women (mean age 37 y), in young pregnant women, and in women who have hormonal excess for any other reason. In most reported cases, nodules either regress or exhibit growth once the hormonal stimulation has been removed."

[But in my case, hormone levels tested normal and I take no hormonal contraceptives!]

"...Leiomyomatosis peritonealis disseminata is found most commonly in women of reproductive age who are pregnant; these patients are usually asymptomatic, have a long-term history of oral contraceptive use, or have uterine leiomyomas at the time of diagnosis. All cases of this disease have been discovered intraoperatively during obstetric and gynecologic surgical procedures."

[All cases? Maybe only the cases these authors looked at. What about the other cases?]

"...Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information. The reader should confirm the information in this article from other sources prior to use..."

Sunday, June 24, 2007

GnRH agonist caused (not cured) LPD!

Changgeng Yi Xue Za Zhi. 1996 Dec;19(4):352-7.
Related Articles,
Links
Leiomyomatosis peritonealis disseminata (LPD) with acute ascites induced by gonadotropin-releasing hormone (GnRH) agonist: a case report।



CW, ChangChien CC, Lin JW, Hsu TY, Chang SY।Department of Obstetrics & Gynecology, Chang Gung Memorial Hospital, Kaohsiung, Taiwan, R।O।C।Leiomyomatosis peritonealis disseminata (LPD) is a rare disorder characterized by the development of numerous leiomyomata throughout the peritoneal cavity। We present a case of a 29-year-old woman who had LPD with acute ascites induced by a GnRH agonist (Supremon; buserelin acetate nasal solution 400 micrograms/per day).

Saturday, March 24, 2007

Mind/Body Connection

My first brush with medical problems, the giant 2001 uterine fibroid, got me investigating alternative therapies and mental connections. I was in pain all the time and was running around desperately looking for answers, yet I couldn’t accept the answers I got. Perhaps my problem was (still is) that I just can’t believe in much more than gravity.

One of the things I think that seems to be wrong with the world, is the idea that “if it works for me, it’ll work for you”. This idea negates our individuality and while it may be a good rule of thumb, it just doesn’t work all the time. We can see this idea manifest when we say, “if that bird-brain can do it, so can I!” or “I’m no genius and did this, so you can too!”. I have a big problem with the idea of role modeling. We shouldn’t live our lives to be an example to others, but be happy with who we are. Likewise, we shouldn’t live our lives following the example of others, but should find our own way.

Even in scientific studies, when treatment x works for, I don’t know – maybe 90% of those studied, it eventually gets put into standard procedure and looked upon as THE cure. What about that 10% for whom it didn’t work? Sometimes there are obvious reasons, but sometimes not.

What I am trying to say here, being of that mysterious non-receptive percentage, is that I am not off the hook: I (me, myself) must keep searching, trial and error, upturning stone after stone and then going back and checking again for what works for me. To complicate matters more, what works at one day, may not work the next and in addition some things may need time to start working and others maybe should just be thrown out right away. How can we tell the difference? There is no savior and the experiences of others are simply their experiences – not predictions of how things will work for me or you.

Friday, March 23, 2007

Decapeptyl 3,75mg

Earlier I mentioned Decopaptyl. It's Decapeptyl. Triptorelina. On the paper that comes in the box it says, "NO SE ACONSEJA UN SEGUNDO TRATAMIENTO CON DECAPEPTY O CON OTRO ANALOGO DE LA LHRH."

That means only one treatment per lifetime for any GnRH Agonist.

Tuesday, March 13, 2007

What is LPD?

"Leiomyomatosis peritonealis disseminata (LPD), also known as diffuse peritoneal leiomyomatosis, is a rare disease in which multiple smooth muscle or smooth muscle-like nodules develop subperitoneally in any part of the abdominal cavity. These nodules, though histologically benign, cannot be distinguished macroscopically from peritoneal carcinomatosis.

The aetiology is thought to be smooth muscle metaplasia of the subperitoneal mesenchyme. About 100 documented cases were found in the English language literature; LPD patients are mainly females of reproductive age, and only rarely have cases affecting men been reported. The condition is associated with high levels of exogenous and endogenous female gonadal steroids (e.g. pregnancy, prolonged exposure to oral contraceptives and/or combined hormonal replacement therapy, granulomatous cell tumours of the ovary), indicating that oestrogens and progestins play an important role in the pathogenesis of LPD as they do in leiomyomata uteri. Most LPD cases are clinically benign, and in some instances the lesions may partially or completely regress . Alternatively, LPD may progress, recur or (rarely) undergo malignant transformation [8]."
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1481579

"Leiomiomatosis Peritoneal Diseminada
La LPD es una enfermedad benigna e infrecuente caracterizada por el desarrollo de múltiples leiomiomas en la cavidad peritoneal. Aparece en mujeres en edad fértil, asociándose con frecuencia a miomas uterinos y/o estados de hiperactividad hormonal (embarazo, anticonceptivos orales, tumores ováricos, etc.); más raramente se ha descrito en mujeres posmenopáusicas no sometidas a terapia hormonal, considerándose esta situación por algunos autores como un diagnóstico tardío de la enfermedad, donde previamente aconteció una situación de hiperestímulo hormonal.

Aunque su etiología es desconocida, hay una clara dependencia hormonal en el desarrollo de esta enfermedad, como lo demuestran las características de la población afectada y la presencia de receptores hormonales en estos tumores. Algunos autores afirman que el origen de los leiomiomas en esta enfermedad se debe a la metaplasia de células multipotenciales con diferenciación miofibrosa del epitelio mülleriano, distribuido durante la embriogénesis en el mesénquima subperitoneal, y que tanto la predisposición individual como el hiperestímulo hormonal serían los factores determinantes de la tumorogénesis.

Las pacientes suelen estar asintomáticas hasta el momento en que se palpa una masa abdominal o aparecen síntomas derivados de la compresión tumoral de órganos de vecindad.

Ante el escaso número de casos y la poca experiencia acumulada, el tratamiento en la bibliografía revisada es muy variable. Hay actitudes muy conservadoras basadas en un seguimiento clinicorradiológico, dada la benignidad del proceso, contrastada por un seguimiento a largo plazo (2, 3 y 10 años) de pacientes en los que no se evidenció degeneración maligna. Por otro lado, otros autores proponen una cirugía radical (histerectomía con doble anexectomía y extirpación de todas las tumoraciones) con el fin de evitar todo influjo hormonal, así como la posibilidad de degeneración maligna, que según diversos estudio varía entre un 3 y un 5%. En nuestro caso optamos por una cirugía radical, debido a la edad de la paciente y la existencia de una dependencia hormonal en el desarrollo de la enfermedad.

Otros autores indican una cirugía radical en mujeres fértiles que no deseen tener más hijos, en mujeres posmenopáusicas y ante la sospecha de progresión o malignización de la enfermedad, intentando resecar el mayor número de tumoraciones. En caso de tumores residuales, puede ser útil un tratamiento hormonal adyuvante mediante acetato de megestrol. En niñas y en mujeres jóvenes sin hijos puede realizarse un seguimiento clinicorradiológico sistemático, no recomendándose el embarazo ni el uso de anticonceptivos orales.
http://db.doyma.es/cgi-bin/wdbcgi.exe/doyma/mrevista.fulltext?pident=13015252

Sunday, March 11, 2007

LPD Treatments

1. Chop them out. They could come back again in the same place or other places. This operation is no walk in the park.
2. Hormone therapy: Remove the source of estrogen with a gnrh agonist such as Decopeptyl combined (or not) with add-back hormones. Heavy-duty as well as new undocumented secondary effects are possible. There are cases of people getting these tumors after hysterectomies and after menopause, so just cutting off the source of the estrogen does not seem to be a definitive solution.
3. No treatment. They are benign after all, but if they are squishing other organs, they compromise the functioning of the entire organism, that’s why they were detected in the first place. Also they sometimes (rarely) transform into cancer.
4. Embolis(z)ation (cut off the feeding vein). This is actually for Uterine Fibroids and I don't think it is an option for LPD. Although it seems to have gone beyond the experimental stage, it is not standard proceedure in this socialized health system so I haven't investigated it very much.
5. Alternative (Holistic, Homeopathy, Herbs, Juicing etc.): No guarantees. If it all worked for everybody all the time, then conventional medicine would never have emerged. See "quackwatch"and the book by reporter-patient John Diamond “Snake Oil And Other Preoccupations”. If it does no harm and conventional medicine has little else to offer, why not? Just watch the pockets. Look for lists of estrogen inhibiting foods and keep away from PABA sunscreens and eating off plastics, try to exercise more, eat salads etc.


Tratamientos
1.Cirugía. Pueden reaparecer en el mismo lugar u otros lugares.
2. Terapia hormonal. Puede ser difícil y nuevos efectos secundarios indocumentados sea posible. Hay casos en que reaparecen estos tumores después de histerectomías y después de menopausia, así que cortar la fuente de estrógeno no se parece ser una solución definitiva para todos.
3. Ningún tratamiento. Son benignas, pero si están aplastando otros órganos, comprometen el funcionamiento del organismo entero, es por eso que fueron detectados en el primer lugar.
4. Embolis(z)ation (cortar la vena de alimentación). Todavía experimental.
5. Tratamientas Alternativas (Holístico, Homeopatía, Hierbas etc.): Ningunas garantías. Si funcionara todo para todos, entonces la medicina convencional nunca habría emergido. Mira a www.quackwatch.com y el libro de John Diamond el reportero-paciente " Snake Oil And Other Preoccupations (aceite de serpientes y otras preocupaciones?)". ¿Si no hace ningún daño y la medicina convencional tiene poco a ofrecer, por qué no? Vigilar el dinero que gastas. La seguridad social aquí es convencional y es gratis. Tengo listas de los alimentos que inhiben estrógenos y no uso sunscreens con PABA y no como de recipientes plásticos, intento ir mas al gimnasio etc..