Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Article
Alveolar soft part sarcoma in pregnancy: A case report and review of the literature

https://www.researchgate.net/profile/Na ... ion_detail

From Olga in 2014

http://www.cureasps.org/forum/viewtopic ... =955#p7287

Alveolar soft part sarcoma in pregnancy: A case report and review of the literature

Discussion

Cancer during pregnancy is rare, affecting only 1 in 1 000-1 500 pregnancies.26 ASPS is rarer still, accounting for only a fraction of all soft tissue sarcomas. The combination is therefore even more unusual. Only one case has been reported in the literature; Tapisiz, et al. reported a woman diagnosed with *lingual ASPS during pregnancy.27 Pregnant patients with malignancy are difficult to manage. Both mother and foetus are at risk. Additionally the usual complete workup and management are complicated. CT scan to diagnose metastatic spread exposes the foetus to excessive and potentially harmful radiation. General anaesthesia for biopsy carries a 1-2% risk of miscarriage and chemotherapy is teratogenic.28 It is important to recognise these potential risks early on and involve a multi-disciplinary team to manage these patients. In our case, the staging CT scan and adjuvant chemotherapy were delayed until after delivery.

Pregnant patients with malignancy are difficult to manage. It is important to recognise potential risks early on and involve a multi-disciplinary team to manage these patients

An interesting finding in this case is the positive progesterone receptor staining. This has been described once previously, in an ASPS case involving the endometrium.29 The authors concluded that the tumour might have originated from endometrial tissue, explaining the finding of progesterone receptors. Most case reports and series in the literature have not specifically evaluated the immunohistochemical expression of hormone receptors in these lesions, even in cases in which lesions developed in sites which contain hormonally responsive tissues. Our patient however had progesterone receptor expression in a lesion arising within skeletal muscle. This might further explain the female predominance in ASPS, and the rapid tumour progression during pregnancy in our patient. Further research into this aspect of ASPS is needed. Most of the tumours previously described in the literature were not associated with pregnancy and the tumour in our case may potentially have been an incidental finding. The presence of hormone receptor expression, however, raises the possibility that this lesion may be related to, or driven by the hormonal milieu related to the underlying gestational state. Hormonal sensitivity was postulated as early as 1988, in a case report describing a patient who developed symptoms related to previously identified lung metastases during pregnancy and the puerperium.30 An additional report from 1960 describes a patient with symptomatic metastases identified during pregnancy, following a pre-pregnancy diagnosis of ASPS.31





http://www.scielo.org.za/scielo.php?scr ... 4000300007

Conclusion

Alveolar soft part sarcoma is an extremely rare tumour of mainly young female patients. The current case is no exception to this pattern, but is unusual in its co-existence with pregnancy and its expression of progesterone receptors, which are of potential therapeutic significance in view of the poor response to current conventional chemotherapy. The latter is a direction for further research, as late and ultimately fatal recurrences are a well-recognised phenomenon of ASPS.

From prior post -

Only one case has been reported in the literature; Tapisiz, et al. reported a woman diagnosed with lingual ASPS during pregnancy
And here is the article -

* Lingual AlveolarAn Unusual Case of Soft Part Sarcoma During Pregnancy

Omer L. Tapisiz'Correspondence information about the author Omer L. TapisizEmail the author Omer L. Tapisiz, Tayfun Gungor, Emin Ustunyurt, Bulent Ozdal, Umit Bilge, Leyla Mollamahmutoglu
Zekai Tahir Burak Womens’ Health Education and Research Hospital, Ankara, Turkey



SUMMARY
Objective: We report a case of alveolar soft part sarcoma (ASPS) of the tongue that presented in the third
trimester of pregnancy.
Case Report: An 18-year-old, gravida 1, para 0, woman with ASPS of the tongue in the 31st week of pregnancy
was described. Approximately 4 months after her first diagnosis, she was referred to our clinic for the delivery
of her baby and additional treatment. At 32 weeks of gestation, a healthy male infant weighing 2,220 g was
delivered by cesarean section. After an uneventful postoperative course, she was referred to the department of
otorhinolaryngology and maxillofacial surgery for further investigation and treatment.
Conclusion: ASPS of the tongue presenting in pregnancy is an extremely rare combination, and to our knowledge,
this is the first reported case in the English literature. The diagnostic and therapeutic management of the pregnant
patient with cancer, like ASPS, is especially difficult, because it involves both the mother and fetus. [Taiwan J Obstet
Gynecol 2008;47(2):212–214]
Key Words: alveolar soft part sarcoma, cancer, pregnancy, tongue


http://www.sciencedirect.com/science/ar ... 2ffb&ccp=y

"The physiologic changes of pregnancy are all potentially
capable of promoting neoplastic growth. The major
reason for suspecting that pregnancy adversely affects
the clinical course of cancer is the immunologic tolerance
that characterizes both conditions. The immune
system is suppressed during the first 20 weeks of gestation,
which in some way predisposes the subject to
malignancy, modifies the biologic behavior of the neoplasm,
and adversely affects the clinical course of the
malignancy [9]. It has been shown that high levels
of estrogen and progesterone during pregnancy may
stimulate latent breast carcinoma to an active state
[10]. However, these hormonal receptors are generally
not found in head and neck tissues, as was the case in
our patient, but the rapid progression of the disease
in our case may be explained by the abovementioned
pregnancy-related physiologic changes. In summary,
humoral factors might act through progesterone and
estrogen receptors, and the immunotolerance characteristic
of pregnancy might also contribute to tumor
progression."

I've been reading that estrogen and progesterone receptors have been linked to the chemosistance tendency of breast cancer.

Asps is also chemoresitant

One more case of the very aggressive ASPS flare up during the last part of the pregnancy term (incl. initial regression of the metastases after delivery, then they are back again in few months incl.heart metastases) is described in an excellent blog of the ASPS patient Brittany Sullivan http://johnandbrittanysullivan.blogspot.ca/ who is currently having an exceptional response to Keytruda+Axitinib on a Dr.Whlky trial. The hormone receptors and the immune tolerance state induced by pregnancy are obvious, IMHO. Progesterone is a hormone that is sharply up during the pregnancy, at delivery, levels of estrogen and progesterone fall, allowing the hormone prolactin to increase and initiate milk production. When the baby was born, it went down and there was regression of her mets, then it went up after some time so the mets appear again....I would try the progesterone blocker, they are avail.!

Efficacy of the Progesterone Receptor Antagonist Mifepristone for Palliative Therapy of Patients with a Variety of Advanced Cancer Types http://ar.iiarjournals.org/content/30/2/623.full
"some types of cancer may ‘borrow’ a mechanism from normal pregnancy that is used to escape natural killer cell surveillance."

Olga
Excellent idea!
The link isn’t working in post above tho

Is this it?
Abstract
https://www.ncbi.nlm.nih.gov/pubmed/20332480/

Full text
http://ar.iiarjournals.org/content/30/2/623.long