Preserving fertility in pseudomyxoma peritonei, a novel approach

AbstractBackgroundCytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) is the gold standard treatment for patients with pseudomyxoma peritonei (PMP) but involves routine bilateral salpingo-oophorectomy. Young women wishing to maintain fertility may be reluctant to pursue this. An alternative strategy in women with low-grade PMP has been explored in the form of laparoscopic evacuation of pelvic and ovarian mucin with resection of the appendiceal tumour.MethodsBetween January 2012 and January 2015, four young women (aged 28–35 years) with PMP seeking to maintain fertility underwent laparoscopy, appendicectomy and pelvic mucinous evacuation and washout. Data regarding intra-operative and histopathological findings were collected. Endpoints were fertility-related outcomes and oncological follow-up.ResultsInfertility was a presenting symptom in three of the four women. All four had significant pelvic mucinous disease on radiological imaging and were offered CRS and HIPEC as definitive treatment, but chose laparoscopy with appendicectomy and copious irrigation and washout of the pelvis with stripping of mucinous disease off the ovarian surfaces. Postoperative histology demonstrated a low-grade appendiceal mucinous neoplasm (LAMN) in all patients with acellular mucin or low-grade mucinous carcinoma peritonei in the peritoneal cavity. All patients successfully conceived subsequently and gave birth to healthy babies. After 12–29 months follow-up, all women are well with no radiological or laparoscopic evidence of disease recurrence.ConclusionsIn patients with low-grade PMP, initial therapeutic laparoscopy can restore fertility, whilst providing short- to medium-term disease control. This modality in young women wishing to have children appears to be a feasible alternative to immediate CRS and HIPEC.

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Patterns of failure after definitive treatment for T4a larynx cancer in the Veterans Affairs Health System.

Journal of Clinical Oncology ◽

10.1200/jco.2021.39.15_suppl.e18006 ◽

2021 ◽

Vol 39 (15_suppl) ◽

pp. e18006-e18006

Author(s):

Rohith S. Voora ◽

Bharat Panuganti ◽

Mitchell Flagg ◽

Abhishek Kumar ◽

Nikhil V. Kotha ◽

...

Keyword(s):

Adjuvant Therapy ◽

Cancer Patients ◽

Disease Recurrence ◽

Distant Recurrence ◽

Larynx Cancer ◽

Definitive Treatment ◽

Lower Probability ◽

Cancer Specific Survival ◽

Curative Intent

e18006 Background: Both chemoradiotherapy (CRT) and total laryngectomy (TL) with adjuvant therapy are curative-intent treatment options for patients with T4a larynx cancer. Disease recurrence is a known negative prognosicator, but differences in recurrence patterns and the subsequent survival associations are not well characterized. To address this knowledge gap, we present long-term recurrence and survival outcomes from a novel longitudinal data source. Methods: Retrospective study of non-metastatic T4a larynx cancer patients diagnosed between 2000-2017 who underwent curative-intent treatment (TL with adjuvant therapy or primary CRT) from the VA Informatics and Computing Infrastructure database. Adjuvant therapy consisted of either postoperative radiotherapy (RT) or CRT. Fine-Gray and Cox models were used to evaluate primary outcomes – time to locoregional recurrence and distant recurrence. Secondary outcomes included overall survival (OS), cancer-specific survival (CSS), non-cancer specific survival (NCSS), and disease-free survival (DFS). These multivariable models accounted for age, race, alcohol history, smoking status, education and income, Charlson-Deyo score, N-classification, and tumor subsite. Results: The study included 1,114 patients with a median follow-up time of 63.3 months among those alive at last follow up. In the TL group, adjuvant RT was used in 69% and adjuvant CRT was used in 31%. Median time to first recurrence was 24.4 months with overall incidence of 28.5% locoregional and 9.5% distant recurrence. Primary CRT patients had higher rates of locoregional (37.2 vs. 22.9%) and distant recurrence (13.3 vs. 7.0%) (p < 0.0001). Median OS was 27.3 months for CRT (95% CI: 23.6-32.4 months) and 47.5 months (95% CI: 39.6-52.1 months) for TL. Median DFS was 14.1 months for CRT (95% CI:12.5-17.2 months) and 37.9 months (95% CI 31.2-47.5 months) for TL. On multivariable analysis compared to CRT, TL was associated with longer time to locoregional (HR 0.50, 95% CI:0.40-0.61) and distant recurrence (HR 0.50, 95% CI:0.34-0.73). Having N+ disease increased risk of distant recurrence (HR 2.20, 95% CI:1.42-3.41). TL was associated with improved OS (HR 0.78, 95% CI:0.67 – 0.91), CSS (HR 0.73, 95% CI:0.59 – 0.89), and DFS (HR 0.58, 95% CI 0.49-0.69) compared to CRT; NCSS was equivalent between groups (HR 1.09, 95% CI:0.88-1.35). Of the CRT patients with locoregional failures, 67/163 (41.1%) were salvaged with surgery. Conclusions: In this cohort of T4a larynx cancer patients, surgical management demonstrated favorable recurrence and survival results. TL with adjuvant therapy was associated with significantly lower incidence of both locoregional and distant recurrence and increased OS, CSS and DFS compared to CRT. Lower probability of disease recurrence, in addition to a survival advantage, should be considered as an important advantage to up-front surgery.

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Unusual Case of Pseudomyxoma Peritonei of a Mature Teratoma in Adult Men

Case Reports and Series in Surgery ◽

10.31487/j.crss.2020.02.04 ◽

2020 ◽

pp. 1-3

Author(s):

Cecilia Ceribelli ◽

M. Gomes David ◽

A. Ayav ◽

J. Raft ◽

...

Keyword(s):

Mitomycin C ◽

Pseudomyxoma Peritonei ◽

Mature Teratoma ◽

Ovarian Teratoma ◽

Low Grade ◽

Adult Men ◽

Crs And Hipec ◽

One Year ◽

Different Origins ◽

Mass Volume

Pseudomyxoma peritonei (PMP) is a rare disease usually originating from appendiceal neoplasm and its incidence is 1-2 cases per million. Extra-appendicular origin is less common and is related to ovary, urachus, stomach, colon and pancreas cancer. A few cases of mature ovarian teratoma associated with PMP have been described. We report an extremely rare case of a man treated for a mature teratoma recurrence as a PMP. A 57-year-old man with multiple surgeries for mature teratoma excision as a newborn was addressed to our institution. At the age of 55, a surgical exploration found a low-grade PMP. The patient was asymptomatic, and surveillance was performed. Nine months later, a sub-complete cytoreductive surgery (CRS) with Mitomycin C HIPEC (hyperthermic intraperitoneal chemotherapy) was performed. One year later, the residual mass volume was treated by a new CRS with Mitomycin C HIPEC. After a two-year follow-up patient has no recurrence. PMP extra-appendiceal origin in less than 10%. This is the first reported case of PMP originating as a mature teratoma recurrence in a man. A few cases describe PMP from ovarian teratoma in women. Despite PMP’s different origins, gold standard treatment still remains CRS and HIPEC.

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Bronchial-Associated Lymphoid Tissue (BALT) Lymphoma: A Multicenter Retrospective Analysis.

Blood ◽

10.1182/blood.v104.11.4583.4583 ◽

2004 ◽

Vol 104 (11) ◽

pp. 4583-4583

Author(s):

Daniele Laszlo ◽

Sara Steffanoni ◽

Giovanna Andreola ◽

Rosaria Balzano ◽

Giancarlo Pruneri ◽

...

Keyword(s):

Systemic Treatment ◽

Radical Surgery ◽

Wedge Resection ◽

Bone Marrow Involvement ◽

Definitive Treatment ◽

Hematological Toxicity ◽

Low Grade ◽

Symptomatic Disease ◽

Balt Lymphoma

Abstract Surgery has been the treatment of choice in patients affected by BALT NHL; however, radical surgery is not always possible and does not avoid the risk of relapse. Systemic treatment is recommended for symptomatic patients who relapse after surgery or cannot undergo such procedure; nevertheless, there is no standard systemic chemotherapy which could be recommended for such indolent disease. Here we report clinical data of 16 patients (9 men and 7 female) with biopsy-proven low grade BALT lymphoma followed at EIO and IOSI from 1992 to 2004. Median age was 62 years (range 50–77); 8 patients presented symptomatic disease at diagnosis. Thoracotomy was necessary to obtain histological material in eight patients, while in the other 8 no-invasive procedures were performed. Thirteen patients were considered in stage IE and 3 in stage IV (bone marrow involvement in 2 patients, gastric in 1), according to Ann Arbor. Five patients received upfront aggressive surgery: 1 underwent wedge resection and 4 lobectomy. Surgery was the definitive treatment for 3 patients. With a median follow-up of 17 months (range 9–31), all these patients are still alive with no evidence of disease. Twelve patients received systemic treatment: two because of non-radical surgery and ten because of advanced stage, symptomatic disease or psychosocial reasons. The majority of them (7 patients) received alkylating containing regimen (Chlorambucil ± PDN), alone or in combination with Rituximab (Mabthera) (2 patients). Three additional patients, refusing CT, received Rituximab alone in a standard schedule. Hematological and non-hematological toxicity was in general mild: transient G3 neutropenia occurred in only one patient. No patient discontinued treatment because of treatment-related toxicity and no toxic death was recorded. All patients responded to systemic therapy with five patients achieving CR. Four of them are still alive and free of disease after 24, 65, 27, 42 months respectively. One patients still in CR died for reasons different from lymphoma after 54 months. With a median follow-up of 60 months (range 31 – 63), only four patients developed progressive disease or relapse after 67, 5, 63, 18 months respectively (median TTP=40 months). Our experience suggests that systemic treatment, when appropriate, is able to achieve an high response rate in this relative uncommon disease. Considering the clinical results and the low degree of toxicity observed, alkylating containing regimen (Chlorambucil ± PDN) alone or in combination with monoclonal antibody could be considered as first line treatment for patients with BALT lymphoma.

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Fertility-sparing treatment in women with endometrial cancer

Clinical and Experimental Reproductive Medicine ◽

10.5653/cerm.2020.03629 ◽

2020 ◽

Vol 47 (4) ◽

pp. 237-244

Author(s):

Seyeon Won ◽

Mi Kyoung Kim ◽

Seok Ju Seong

Keyword(s):

Endometrial Cancer ◽

Young Women ◽

Eligible Patient ◽

Myometrial Invasion ◽

Definitive Treatment ◽

High Dose ◽

Low Grade ◽

Endometrioid Adenocarcinoma ◽

Fertility Sparing ◽

The Ideal

Endometrial cancer (EC) in young women tends to be early-stage and low-grade; therefore, such cases have good prognoses. Fertility-sparing treatment with progestin is a potential alternative to definitive treatment (i.e., total hysterectomy, bilateral salpingo-oophorectomy, pelvic washing, and/or lymphadenectomy) for selected patients. However, no evidence-based consensus or guidelines yet exist, and this topic is subject to much debate. Generally, the ideal candidates for fertility-sparing treatment have been suggested to be young women with grade 1 endometrioid adenocarcinoma confined to the endometrium. Magnetic resonance imaging should be performed to rule out myometrial invasion and extrauterine disease before initiating fertility-sparing treatment. Although various fertility-sparing treatment methods exist, including the levonorgestrel-intrauterine system, metformin, gonadotropin-releasing hormone agonists, photodynamic therapy, and hysteroscopic resection, the most common method is high-dose oral progestin (medroxyprogesterone acetate at 500–600 mg daily or megestrol acetate at 160 mg daily). During treatment, re-evaluation of the endometrium with dilation and curettage at 3 months is recommended. Although no consensus exists regarding the ideal duration of maintenance treatment after achieving regression, it is reasonable to consider maintaining the progestin therapy until pregnancy with individualization. According to the literature, the ovarian stimulation drugs used for fertility treatments appear safe. Hysterectomy should be performed after childbearing, and hysterectomy without oophorectomy can also be considered for young women. The available evidence suggests that fertility-sparing treatment is effective and does not appear to worsen the prognosis. If an eligible patient strongly desires fertility despite the risk of recurrence, the clinician should consider fertility-sparing treatment with close follow-up.

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Posterior pituitary tumours: patient outcomes and determinants of disease recurrence or persistence

Endocrine Connections ◽

10.1530/ec-20-0621 ◽

2021 ◽

Author(s):

Liza Das ◽

Kim Vaiphei ◽

Ashutosh Rai ◽

Chirag Kamal Ahuja ◽

Paramjeet Singh ◽

...

Keyword(s):

Literature Review ◽

Tumour Size ◽

Disease Recurrence ◽

Low Grade ◽

Posterior Pituitary ◽

Pituitary Tumours ◽

Thyroid Transcription Factor 1 ◽

S 100

Objective: Posterior pituitary tumours (PPTs) are rare neoplasms with the four recognised subtypes unified by thyroid transcription factor-1 (TTF-1) expression, according to the 2017 WHO classification. Though traditionally defined as low grade neoplasms, a substantial proportion of them show recurrence/persistence following surgery. Methods: We selected patients with PPTs in our cohort of 1760 patients operated for pituitary tumours over the past ten years. The clinical, radiological, hormonal, histopathological profiles and long-term outcomes of the three cases identified (two pituicytomas and one spindle cell oncocytoma, SCO) were analysed. Following a literature review, data of all published cases with documented TTF-1 positive pituicytomas and SCOs were analysed to determine the predictors of recurrence/persistence in these tumours. Results: Patients presented with compressive features or hypogonadism. Two had sellar-suprasellar masses. One had a purely suprasellar mass with a pre-operative radiological suspicion of pituicytoma. Two were operated by transsphenoidal surgery and one transcranially guided by neuronavigation. Histopathology confirmed spindle cells in a storiform arrangement and low Ki67 index. Immunohistochemistry showed positive TTF-1, S-100 expression and variable positivity for EMA, vimentin and GFAP. Re-evaluation showed recurrence/persistence in two patients. A literature review of recurrent/persistent pituicytoma (n=17) and SCO (n=9) cases revealed clinical clues (headache for pituicytomas, male gender for SCO), baseline tumour size (≥20.5mm with sensitivity exceeding 80%) and longer follow-up duration as determinants of recurrence/persistence. Conclusion: PPTs are rare sellar masses with quintessential TTF-1 positivity. Recurrent/persistent disease following surgery is determined by greater tumour size at baseline and duration of follow-up. This warrants intensive and long-term surveillance in these patients.

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Computed Tomography Follow-Up Assessment of Patients with Low-Grade Appendiceal Mucinous Neoplasms: Evaluation of Risk for Pseudomyxoma Peritonei

Annals of Surgical Oncology ◽

10.1245/s10434-016-5623-3 ◽

2017 ◽

Vol 24 (7) ◽

pp. 1778-1782 ◽

Cited By ~ 11

Author(s):

Catarina Tiselius ◽

Csaba Kindler ◽

Jayant Shetye ◽

Henry Letocha ◽

Kenneth Smedh

Keyword(s):

Computed Tomography ◽

Pseudomyxoma Peritonei ◽

Low Grade ◽

Mucinous Neoplasms

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Pulmonary Metastasis from Pseudomyxoma Peritonei

Gastroenterology Research and Practice ◽

10.1155/2012/690256 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 10

Author(s):

Toshiyuki Kitai

Keyword(s):

Pulmonary Metastasis ◽

Pseudomyxoma Peritonei ◽

Clinical Findings ◽

Therapeutic Modality ◽

Locoregional Therapy ◽

Rapid Progression ◽

Low Grade ◽

Abdominal Lesions ◽

Disease Free

Pseudomyxoma peritonei (PMP) is a rare clinical condition, where copious mucinous ascites accumulate in the peritoneal cavity due to dissemination of mucin-producing tumor. Because of this disseminating, yet nonmetastasizing, behavior, PMP attracts much interest from surgical oncologists in that aggressive locoregional therapy can give the opportunity of long survival and even cure. Although extra-abdominal metastasis is exceptionally rare, the lung is the most likely site in such a case. In this paper, the clinical findings and treatment of eleven cases with pulmonary metastasis from PMP were reviewed, including ten cases in the literature and one case which we experienced. The clinical features of PMP cases with pulmonary metastasis were similar to cases without pulmonary metastasis. The histological type was low-grade mucinous neoplasm in most cases. Pulmonary lesions were resected in seven cases in which abdominal lesions were controlled by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy or another therapeutic modality. Disease-free state was maintained in five cases at the end of the follow-up period. However, it should be noted that rapid progression after resection was seen in two cases, suggesting that biological features may have changed by surgical intervention.

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Recurrence of Pseudomyxoma Peritonei within the Inguinal Canal

Tumori Journal ◽

10.1177/030089160509100607 ◽

2005 ◽

Vol 91 (6) ◽

pp. 481-486 ◽

Cited By ~ 1

Author(s):

Jacobo Cabanas ◽

Rodrigo Gomes da Silva ◽

Paulo Goldstein ◽

Mohan Verghese ◽

Paul H. Sugarbaker

Keyword(s):

Inguinal Hernia ◽

Cytoreductive Surgery ◽

Pseudomyxoma Peritonei ◽

Disease Recurrence ◽

Inguinal Region ◽

Definitive Treatment ◽

Mucinous Tumor ◽

Hernia Sac ◽

Prior Surgery ◽

Common Clinical Presentation

Aim and Background Tumor appearing in an inguinal hernia sac indicates widespread carcinomatosis with ascites. A new onset hernia is a common clinical presentation of pseudomyxoma peritonei syndrome arising in an appendiceal mucinous tumor. Recurrence of pseudomyxoma peritonei within the inguinal region was previously reported in only a single patient. We present five patients with a recurrence of pseudomyxoma peritonei tumor nodules in the inguinal region following cytoreductive surgery and attempt to understand the etiology of this treatment failure. Methods and Study Design From a database of 910 patients with mucinous appendiceal tumors with peritoneal dissemination who had definitive treatment, five patients who had disease recurrence within the inguinal region were identified. The clinical histories were studied and presented in order to make future recommendations regarding the management of these patients. Results Five patients with ages ranging 39 to 67 years had a mucinous tumor in the inguinal region as a recurrence after a previous cytoreductive surgery combined with perioperative intraperitoneal chemotherapy. One of them had separate recurrence in both right and left inguinal regions. All these patients were made disease-free by the reoperative surgery with a sacrifice of the testicle in four. None have evidence of a recurrence of an inguinal hernia despite the lack of formal repair. Conclusion At the time of initial cytoreductive surgery the surgeon should be aware of the inguinal region as a possible site of relapse. This may be more problematic in patients who have had prior surgery with mucoid fluid in a hernia as the presenting sign of pseudomyxoma peritonei. Inguinal recurrences should be removed with clear margins even if orchiectomy is required.

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Fertility-Preserving Treatment in Young Women With Endometrial Adenocarcinoma: A Long-Term Cohort Study

International Journal of Gynecological Cancer ◽

10.1097/igc.0000000000000098 ◽

2014 ◽

Vol 24 (4) ◽

pp. 718-728 ◽

Cited By ~ 19

Author(s):

Chin-Jung Wang ◽

Angel Chao ◽

Lan-Yan Yang ◽

Swei Hsueh ◽

Yu-Ting Huang ◽

...

Keyword(s):

Endometrial Carcinoma ◽

Young Women ◽

Endometrial Adenocarcinoma ◽

Myometrial Invasion ◽

Megestrol Acetate ◽

Published Data ◽

High Dose ◽

Low Grade

ObjectiveGrowing evidence suggests that fertility-preserving treatment is feasible for young women with early-stage, low-grade endometrial carcinoma. However, published data on their long-term outcomes and prognostic factors remain scanty. We aimed to investigate the outcomes of young women receiving fertility-preserving treatment.MethodsBetween 1991 and 2010, the outcomes of young women with grade 1 endometrioid endometrial carcinoma at presumed stage IA (without myometrial invasion) who underwent fertility-preserving treatment of megestrol acetate 160 mg/d with or without other hormonal agents were retrospectively analyzed.ResultsWe identified 37 eligible patients (median age, 32 years; range, 18–40 years). The median follow-up time was 78.6 months (range, 19.1–252.8 months). Complete response (CR) lasting more than 6 months was achieved in 30 (81.1%) women. Responders were significantly younger than nonresponders (P= 0.032). Of the 30 women who had a CR, 15 (50.0%) had disease recurrence. The 5-, 10-, and 15-year cumulative recurrence-free survival rates were 51.0%, 51.0%, and 34.0%, respectively. Notably, those recurred were significantly older (P= 0.003), and the time to CR was significantly longer (P= 0.043) than those without recurrence. One patient developed late recurrences at 156 months, and 2 patients developed ovarian metastasis (6 and 137 months from diagnosis). All the patients are currently alive.ConclusionsThis study demonstrates the feasibility of high-dose megestrol acetate–based therapy for fertility preservation. The substantial risk of late recurrences highlights the need for long-term follow-up studies of large sample sizes with in-depth tumor and host molecular signatures.

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Radical radiotherapy for localized prostate cancer in elderly

Acta chirurgica iugoslavica ◽

10.2298/aci0504103s ◽

2005 ◽

Vol 52 (4) ◽

pp. 103-107

Author(s):

S. Stojanovic ◽

Lj. Radosevic-Jelic ◽

C. Tulic ◽

I. Popov ◽

D. Babic ◽

...

Keyword(s):

Prostate Cancer ◽

Overall Survival ◽

Elderly Patients ◽

Local Control ◽

High Energy ◽

Definitive Treatment ◽

Low Grade ◽

Radical Radiotherapy ◽

Disease Free

Background: Prostate cancer is an age related neoplasm, with high incidence in the group of elderly man. The correct management must to be balanced between the benefits of the treatment and its disadvantages. Radiotherapy as definitive treatment is commonly reserved for older patients and patients with high surgical risk and is widely used as a treatment of choice. Aim: The aim of this study was to determine the role of radical radiotherapy for localized prostate carcinoma in patients 70 years age or older concerning treatment morbidity, local control, disease free and overall survival. Material and methods: A clinical prospective non-randomized study was performed including 103 elderly patients with an age 70 or above, between January 1991 and April 2005, at the Institute for Oncology and Radiology of Serbia. Median age of patients was 74,89 years (range 70- 80 years). Stage distribution was as follows: stage A - 3 patients (2.9 %), stage B - 69 patients (67%) and stage C - 31 patients (30. l%). Out of 103 patients, initial PSA value was noted in 87 patients. The mean value of initial PSA was 18,06 ng/ml. Radical radiotherapy was conducted on megavoltage linear accelerators with high energy photons (10, 18 MeV) and total tumor dose of 65 Gy. Results: Low grade acute complications were registered in 70 patients (65%). Mean follow up time was 40, 13 months. The disease outcome at the last follow up show that 79 patients (76.7%) had no evidence of disease and 24 patients (23.3%) relapsed. Overall survival rates were 65, 29% and 44, 52% and disease free survival 66, 59% and 63, 26% at 5 and 10 years. Disease specific survival was at 5 and 10 years 73,32% and 65, 42% respectively. Late sequels (gradus I and II) are registered in 22 patients (21.36%), out of 103. Conclusion: Radical radiotherapy for localized carcinoma of the prostate is effective treatment option in elderly patients with good local control, present treatment tolerance providing good quality of life and long term cure.

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