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Leiomyosarcoma of the inferior vena cava is a rare tumor that presents in an insidious manner with non-specific symptoms. Given its rarity, there are no consensus guidelines to its management. The aim of this study was to report the clinical experience in the management of patients presenting to our institution during a 12 year period. Four patients with leiomyosarcomas of the inferior vena cava were managed at our institution during the period reviewed. Patient details were identified through a search of the pathology department computerized database, and case notes were retrospectively reviewed to obtain details of presentation and management. There were 3 females and 1 male with a mean age of 59 years. All tumors were identified within 2 months of first symptoms. Three of the 4 had localized tumors whilst 1 patient had lung metastases at presentation. The three patients with resectable tumors underwent radical surgical excision of the tumor, and two patients had postoperative radiotherapy. One patient died of recurrence at 7 months, and another at 30 months. The third patient is currently well and disease free at 16 months. The fourth patient with metastatic disease was treated with chemotherapy alone and survived 36 months. Leiomyosarcoma of the inferior vena cava is an uncommon tumor that presents with non-specific symptoms. At the time of presentation, tumors are usually large and resection is challenging but probably offers the best opportunity for long-term survival.
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BioMed Central
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World Journal of Surgical Oncology
Open Access
Case report
Leiomyosarcoma of the inferior vena cava: Clinical experience with
four cases
Said Abisi
1
, Gareth J Morris-Stiff
1
, David Scott-Coombes
1
, Ian M Williams
1
,
Anthony G Douglas-Jones
2
and Malcom C Puntis*
1
Address:
1
Department of Surgery, University Hospital of Wales, Heath Park, Cardiff, CF14 4XN, UK and
2
Department of Pathology, University
Hospital of Wales, Heath Park, Cardiff, CF14 4XN, UK
Email: Said Abisi - said.abisi@kcl.ac.uk; Gareth J Morris-Stiff - garethmorrisstiff@hotmail.com; David Scott-Coombes - David.Scott-
Coombes@cadiffandvale.wales.nhs.uk; Ian M Williams - Ian.Williams@cadiffandvale.wales.nhs.uk; Anthony G Douglas-Jones - douglas-
jones@cf.ac.uk; Malcom C Puntis* - Malcolm.Puntis@cadiffandvale.wales.nhs.uk
* Corresponding author
Abstract
Background: Leiomyosarcoma of the inferior vena cava is a rare tumor that presents in an
insidious manner with non-specific symptoms. Given its rarity, there are no consensus guidelines
to its management. The aim of this study was to report the clinical experience in the management
of patients presenting to our institution during a 12 year period.
Patients and Methods: Four patients with leiomyosarcomas of the inferior vena cava were
managed at our institution during the period reviewed. Patient details were identified through a
search of the pathology department computerized database, and case notes were retrospectively
reviewed to obtain details of presentation and management.
Results: There were 3 females and 1 male with a mean age of 59 years. All tumors were identified
within 2 months of first symptoms. Three of the 4 had localized tumors whilst 1 patient had lung
metastases at presentation. The three patients with resectable tumors underwent radical surgical
excision of the tumor, and two patients had postoperative radiotherapy. One patient died of
recurrence at 7 months, and another at 30 months. The third patient is currently well and disease
free at 16 months. The fourth patient with metastatic disease was treated with chemotherapy alone
and survived 36 months.
Conclusion: Leiomyosarcoma of the inferior vena cava is an uncommon tumor that presents with
non-specific symptoms. At the time of presentation, tumors are usually large and resection is
challenging but probably offers the best opportunity for long-term survival.
Background
Leiomyosarcomas of the inferior vena cava (IVC) are rare
as illustrated by the fact that an International IVC Leiomy-
osarcoma registry established in 1992 collected, by means
of literature review and personal communication, only
218 cases by the time of publication of its first report in
1996 [1]. Given their anatomical location, it is not surpris-
ing that they often present insidiously with non-specific
symptoms such as: abdominal pain, weight loss, mass,
fever, weakness, anorexia, vomiting, night sweats, dysp-
Published: 04 January 2006
World Journal of Surgical Oncology 2006, 4:1 doi:10.1186/1477-7819-4-1
Received: 28 September 2005
Accepted: 04 January 2006
This article is available from: http://www.wjso.com/content/4/1/1
© 2006 Abisi et al; licensee BioMed Central Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0
),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
World Journal of Surgical Oncology 2006, 4:1 http://www.wjso.com/content/4/1/1
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noea, or they may present with Budd-Chiari syndrome
[2,3].
IVC Leiomyosarcomas are classified anatomically accord-
ing to their relationship to the liver and renal vessels:
lower or Segment I (below the renal vessels); middle or
Segment II (renal vessels to retrohepatic IVC) and upper
or Segment III (suprahepatic IVC) [4]. The management
of these tumors has been, where possible, by means of
radical en-bloc resection with a view of achieving clear
margins. However, in spite of an overall 50% 5-year sur-
vival, half of the tumors recur either locally or metastasize
[1,3].
We present our experience with four cases of IVC leiomy-
osarcoma managed at our institution between January
1993 and December 2004.
Table 1: Demographic features and presenting symptoms of patients with IVC leiomyosarcoma.
Patients
1234
Demographics
Sex Female Female Male Female
Age57605761
Symptoms & Signs
Abdominal Pain Yes Yes No Yes
Weakness No Yes Yes Yes
Anorexia No Yes No Yes
Vomiting No No No Yes
Night sweats No No No No
Dyspnoea No No No No
Mass No No No No
FeverNoNoNoNo
Abdominal wall veins
distension
YesNoNoNo
Diagnosis
Modalities used USS + CT USS + CT CT USS + CT
Location Lower Middle Upper Middle
Size 8 × 14 cm 8 × 7 cm 10 × 10.5 cm 9.5 × 7 cm
Embolus present No No Yes No
Metastasis present Yes (Lung) No No No
Biopsy performed Yes No No No
USS – ultrasound scan; CT – computed tomography
Table 2: Management and outcome data for patients with IVC leiomyosarcoma.
Patients
1234
Management
Operation No Resection and IVC PTFE
Patch
Resection and primary
repair
Resection and IVC Vein
Patch
ChemotherapyYesNoNoNo
Adjuvant therapy None Radiotherapy None Radiotherapy
Histological grade High High High High
Complications
Limb oedema NA No No No
Venous insufficiency NA No No No
Other None No CVA No
Follow-up
Recurrence - No Yes (5 months) No
Status Dead Dead Dead Alive
Survival 36 months 30 months 7 months 16 months
NA – not applicable; CVA – cerebrovascular accident
World Journal of Surgical Oncology 2006, 4:1 http://www.wjso.com/content/4/1/1
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Patients and methods
From January 1993 to December 2004, 4 patients were
diagnosed with leiomyosarcomas of the IVC. There were 3
females and 1 male with a mean age of 59 years (range:
57–61 years). The demographic details and symptoms at
presentation are summarized in Table 1.
Clinical data was obtained from review of the patient
notes, whilst radiological and histological results were
obtained from prospectively maintained departmental
computerized databases.
Results
All 4 patients were symptomatic at presentation although
there was significant variation in symptoms between
patients (Table 1). Patient 1 was referred specifically for
investigation of distended abdominal wall veins.
The diagnosis of a caval tumor was made on an ultra-
sound scan (USS) in 3 of the 4 cases and on computed
tomography (CT) in the fourth. CT scans were performed
to further characterize the origin of the tumors, demon-
strate any contiguous invasion, and to exclude extra-
abdominal metastases (Figure 1). Patient 3 was diagnosed
with a tumor embolus to the right atrium preoperatively.
No further preoperative imaging was performed. Given
the location of the tumors, and based on experience with
other sarcomas, no biopsy was performed preoperatively
for the 3 patients undergoing surgical excision. Patient 1
underwent biopsy of the primary tumor confirm the diag-
nosis prior to commencement of chemotherapy.
Computerized tomographic scan of patient 1 demonstrating a large tumorFigure 1
Computerized tomographic scan of patient 1 demonstrating a large tumor.
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Of the patients who underwent laparotomy, the tumor
was excised en-bloc in all 3 cases. Patient 3 also underwent
a thoracotomy and excision of a tumor embolus from the
right atrium. In this patient, the point of origin of the
tumor on the IVC was narrow and there was enough
mobility to primarily close the IVC. Whilst in the remain-
ing 2 cases, the tumor had a broad base. In these cases, the
tumor was excised with a generous cuff of normal IVC
wall, the defect was patched with a portion of long saphe-
nous vein in patient 4 (Duplex ultrasound was used pre-
operatively to demonstrate the absence of deep venous
thromboses, and deep venous incompetence in the lower
limbs. In-patient 2 a PTFE patch was used to repair the IVC
defect.
All tumors were large volume masses and had the classical
tan-colored, nodular appearance (Figure 2). Histological
examination revealed all to be high-grade leiomyosarco-
mas composed of spindle cells with positive immunohis-
tochemistry staining for Desmin, HHF 35(muscle specific
actin), SMA (smooth muscles actin), and negative for epi-
thelial marker AE1/AE3, S100 protein confirming smooth
muscles origin, and excluding nerve sheath origin (Figure
3).
The postoperative course was unremarkable for 2 patients.
Patient 3, however, suffered a cerebrovascular accident
perioperatively, but subsequently made a full recovery.
Importantly, there were no complications in relation to
the venous blood flow in the inferior vena cava and no
renal impairment. Two of the patients (patient 2 and 4)
undergoing resection received postoperative radiother-
apy.
Patient 1 had a variety of chemotherapy protocols as well
as palliative therapy, and survived 36 months. Disease
recurrence occurred in patients 2 and 3. Patient 3 pre-
sented with widespread metastases and died at 7 months,
whilst patient 2 developed disseminated disease 2 years
after his surgery and died at 30 months post resection.
Patient 4 is alive and disease free at 16 months post sur-
gery.
Gross appearance of the tumorFigure 2
Gross appearance of the tumor.
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Discussion
The first reported case of leiomyosarcoma of the inferior
vena cava was documented in the German literature by
Perl [5] in 1871 but as of 1996 only 218 had been col-
lected [1]. Although only 2% of leiomyosarcomas are vas-
cular in origin, tumors of the IVC account for at least half
the cases [4]. The majority of cases (75–90%) occur in
women [4].
Leiomyosarcomas of the IVC are rare malignant tumors
originating from the smooth muscle cells of the media of
the cava and demonstrate three growth patterns – extralu-
minal, intraluminal or both [6]. The tumors are encapsu-
lated, consisting of lobulated whorls, and histological
examination demonstrates typical spindle-shaped bun-
dles of cells with high mitotic activity and positive stain-
ing for desmin, HHF35, vimentin and smooth muscle
actin.
This tumor commonly presents with a few months history
of vague upper abdominal symptoms including pain,
anorexia and vomiting; but may also present with more
distinct signs such as an abdominal mass, deep venous
thrombosis due to IVC occlusion, or less commonly as an
acute Budd-Chiari Syndrome. However metastasis may be
the first presentation of the tumor such as in case 1.
Given the rarity of the tumor, and in the absence of symp-
toms and signs of IVC compression, the identification of
an IVC leiomyosarcoma is often an unexpected finding on
imaging with USS or CT. In 3 of the 4 cases in this series,
the tumor was identified on USS and a CT scan was per-
formed to determine the origin, evaluate any local inva-
sion of the tumor and exclude pulmonary metastases. In
all 3 cases undergoing surgery, a diagnosis of leiomyosar-
coma was suspected preoperatively. Other imaging
modalities that aid the diagnosis of IVC sarcomas include
vena cavography, which allows biopsy of the tumor. Nev-
ertheless, in some cases, the precise origin of the tumor
cannot be confirmed preoperatively. Furthermore, percu-
taneous biopsy to confirm histology of tumors in this
region is often not possible due to the anatomical location
of the IVC. Thus the surgeon is often faced with a tumor
of uncertain type and origin at laparotomy.
Whilst there is no strong evidence base for the manage-
ment of IVC leiomyosarcomas, the treatment of choice
based on the available literature is radical en-bloc surgical
Photomicrograph of the edge of the tumour (× 40)Figure 3
Photomicrograph of the edge of the tumour (× 40). (A). The tumour is composed of spindle cells which show nuclear
pleomorphism with occasional giant nuclei (Hematoxylin and eosin × 200), (B). Immunohistochemistry for epithelial marker
AE1/AE3 (C), and for S100 (D) is negative, but tumor is strongly positive for smooth muscle marker smooth muscle actin(E).
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excision with a view to obtaining negative resection mar-
gins. A number of techniques have been reported for deal-
ing with the IVC following excision including IVC
ligation; primary repair of IVC; patching of IVC and inter-
position grafting with a synthetic conduit. In the largest
single institute series of 25 patients over a 20 year period,
Hollenbeck et al [7], concluded that primary repair was
the method of choice where applicable as this was associ-
ated with the lowest complication rate. In our limited
experience, patching of the IVC with a segment of long
saphenous vein allowed adequate tumor resection with-
out constricting the IVC. In the single case in which a pri-
mary repair was performed, tumor recurred although it is
uncertain as to whether a wider resection and caval recon-
stitution would have changed the outcome.
When radical surgery is performed, 5- and 10-year results
of 49.4% and 29.5% respectively have been reported [1].
However, recurrence rates are as high as 50% and thus sur-
gery may simply be providing palliation in many cases
[3]. Tumors of the retrohepatic IVC tend to present earlier
as a result of pressure exerted on surrounding structures
and as such have a superior prognosis with 5- and 10-year
survival rates of 56.7% and 47.3% [1]. Other favorable
factors identified by the registry include abdominal pain,
the presence of a mass and the ability to achieve clear
resection margins whereas poor prognostic indicators
include high-grade tumors, suprahepatic tumors, and
presentation with IVC occlusion or Budd-Chiari syn-
drome.
The role of chemotherapy and radiotherapy in the treat-
ment of IVC leiomyosarcoma, either as a primary modal-
ity or as an adjuvant, remains to be proven. In this series,
different chemotherapy protocols provided good pallia-
tion for a patient presenting with advanced disease. In the
remaining three patients, only two underwent radiother-
apy with a view to obtaining local control and prevention
of local recurrence.
Conclusion
Whilst IVC leiomyosarcomas are certainly rare, long-term
survival depends on obtaining an early diagnosis and per-
forming extensive surgery. The more frequent use of CT in
the investigation of patients with abdominal symptoms
may aid the earlier diagnosis of these tumors.
Competing interests
The author(s) declare that they have no competing inter-
ests.
Authors' contributions
SA: writing the article and literature review
GJM: writing the discussion and searching the literature
DS: operative procedure and care of one of the studied
cases
IW: reviewing the article and operative procedure and care
of one of the studied cases
AGD: contributing the pathological aspects of the manu-
script
MCP: overall supervision of the article writing
Acknowledgements
The fourth patients consent was obtained for publication of this case
report.
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... 4,[5][6][7][8][9][10] According to the location, leiomyosarcomas of the IVC can be classified into three categories: (i) lower segment, which extends from the common iliac veins in the lower vena cava to the region close to the renal vessels (34% of leiomyosarcomas occur in this segment); (ii) infra-renal or middle segment, which occurs between the renal and hepatic veins (42% of leiomyosarcomas cases occur in this segment); and (iii) the upper segment, which extends from the hepatic veins to the right atrium of the heart (24% of leiomyosarcomas occur in these segments). [11][12][13][14] Involvement of the upper segment might manifest as Budd-Chiari syndrome, middle segment involvement might manifest as nephrotic syndrome, and involvement of the lower segment might manifest with edema of the lower extremities. The most common symptom is mild, non-specific, abdominal pain, usually preceding diagnosis by months or even years. ...
... Leiomyosarcomas are slow-growing tumors and, often present with insidious and non-specific symptoms, such as abdominal pain, weight loss, palpable abdominal mass, weakness, fever, anorexia, vomiting, and night sweats, due to their retroperitoneal location. 11,12,14,15 Uncommon complications include pulmonary embolism, anemia, depletion of clotting factors, and cardiac arrhythmias. Due to this disease, additional metastatic invasion to adjacent organs, such as the kidneys and liver, can occur. ...
... The tumor can also involve more than one segment, which generates a combination of signs and symptoms. [9][10][11] Imaging contributes significantly to diagnosing and treating leiomyosarcomas. Regarding diagnostic imaging tests, ultrasonography has low diagnostic accuracy, but it can be used to rule out hepatobiliary and pancreatic disorders, and to confirm the presence of the tumor mass. ...
Article
Full-text available
Leiomyosarcoma is a malignant tumor that develops from the smooth muscle constituents of the middle layer in veins, usually affecting the inferior vena cava (IVC); however, this disease is observed in older patients. In this report, we describe a rare case of a 32-year-old female patient who presented with leiomyosarcoma. Initially, the patient complained of moderate-intensity pain in the abdominal region of the right hypochondrium and nausea, worsening postprandial starting 6 days earlier. Abdominal ultrasonography showed a heterogeneous hypoechoic expansive formation, with lob-ulated contours and ill-defined limits in the projection of the IVC. Inferior vena cavog-raphy, showed obliteration of the vessel in almost all of its extensions. Magnetic resonance examination showed a large expansive lesion occupying the IVC. The lesion was resected, and the specimen was sent for anatomopathological analysis, which showed a leiomyosarcoma of the IVC. Patients with leiomyosarcoma have a poor prognosis, and early diagnosis and complete resection with free surgical margins are the only chance of long-term survival. The report stands out due to the appearance of this condition in a young patient with no family history of this disease or other predisposing carcinogenic pathologies. K E Y W O R D S abdominal neoplasm, abdominal pain, diagnostic imaging, leiomyosarcoma, radiology, vena cava
... nal mass, metastatic disease may be the first manifestation of the tumor [3] . ...
... Leiomyosarcoma of the vena cava is classified on the bases on their relation to the liver and the renal vessels into 3 types: lower tumors or Segment I tumors which are located below the renal vessels, middle tumors or Segment II tumors which are located in the region between the renal vessels to retrohepatic inferior vena cava, and upper tumors or Segment III tumors which are located in the supra-hepatic portion of the inferior vena cava [3] . ...
... The management options are tailored toward the individual cases; this may include complete enbloc surgical excision. After resection there are many techniques that are used to deal with the inferior vena cava which may include ligation of the inferior vena cava, primary repair, patch repair, and synthetic interposition graft [3] . ...
Article
Full-text available
a b s t r a c t Primary tumors arising from the inferior vena cava are extremely rare, leiomyosarcoma is the most common one arising from the smooth muscle cells in the media of the wall of the vena cava. A 42-year-old lady had epigastric pain and back pain for 4 months with signs of deep vein thrombosis of the right lower limb. CT-scan showed a mass related to the inferior vena cava which was proved by histopathological examination to be leiomyosarcoma of the inferior vena cava. A multidisciplinary team is required for the diagnosis and management of tumors the vena cava. Long term follow-up is recommended.
... The inferior vena cava leiomyosarcoma (IVC) is a rareneoplasm. Up to now, approximately 300 cases have been reported since the first autopsy diagnosis (3,4). This tumor originates from the smooth muscle cells of the IVC tunica media. ...
... Tumor clinic depends basically on its location and size (1,2). But its growth is insidious with very specific symptoms such as abdominal pain, weight loss, abdominal tumors, fever, weakness, anorexia, vomiting, night sweats and eventually with Budd-Chiari syndrome (2,4,6,7). ...
... The best treatment, with impact on survival, is complete surgical resection of the tumor without compromising the surgical margins (2,4,6). Treatment options following resection of IVC leiomyosarcomas include primary repair, ligature or reconstruction with prosthesis and depend on the location of the tumor and invasion of adjacent organs and vessels, especially of the renal veins (5) This article reports a rare case that was addressed at the Vascular Surgery Service of the Samuel Libânio Clinic Hospital in Pouso Alegre, MG, which should be included in the differential diagnosis of retroperitoneal masses, since its surgical planning has an impact on patient survival ...
Article
Full-text available
The inferior vena cava leiomyosarcoma (IVC) is a rareneoplasm. It originates from the smooth muscle cells of the IVC tunica media. The tumor's clinic depends basically on its location and size. The best treatment, with impact on survival, is complete surgical resection.
... Even with radical surgery, 5-and 10-year survival of 49.4% and 29.5% respectively has been reported [19]. With recurrence rates as high as 50% reported in some literature, surgery may simply be providing palliation in many cases [19]. ...
... Even with radical surgery, 5-and 10-year survival of 49.4% and 29.5% respectively has been reported [19]. With recurrence rates as high as 50% reported in some literature, surgery may simply be providing palliation in many cases [19]. Tumors of the retrohepatic IVC tend to present earlier as a result of pressure exerted on surrounding structures. ...
... Tumors of the retrohepatic IVC tend to present earlier as a result of pressure exerted on surrounding structures. A superior prognosis with 5-and 10-year survival rates of 56.7% and 47.3% are reported [19]. Abdominal pain, the clinical presence of a mass and the ability to achieve clear resection margins were noted to be favorable factors whereas poor prognostic indicators include high-grade tumors, suprahepatic tumors, and ...
Article
Full-text available
Leiomyosarcomas of the Inferior Vena Cava (IVC) are rare tumors accounting for only 0.5% of all soft tissue sarcomas in adults with fewer than 300 cases reported. Intraluminal leiomyosarcoma of IVC is rare. The involvement of renal or hepatic veins determines the strategy for vascular reconstruction. Recent studies have shown that radical surgery with adjuvant multimodal therapy improves the cumulative survival rate. We report a case of IVC leiomyosarcoma in a young healthy woman along with diagnostic workup followed by resection of tumor and reconstruction of IVC by Polytetrafluoroethylene (PTFE) graft. She reports no recurrence/complications after 6 cycles of chemotherapy and 40 months of follow-up. The prognosis depends on grade of the tumor, segment of the IVC involved (level 2-better prognosis), size of tumor (size<9 cm better prognosis) and adjuvant therapy. However, overall leiomyosarcoma runs a poor prognosis; over half of the patients with surgical excision develop recurrence. The 5-year survival rate for these patients ranges between 31 and 62%.
... 1,2 O leiomiossarcoma da veia cava inferior (VCI) é uma neoplasia rara originada das células do músculo liso da túnica média, sendo relatados, até o momento, aproximadamente 300 casos no mundo desde o primeiro diagnóstico de autópsia feito por Virchow e descrito por Perl em 1871. 1,3,4 A primeira ressecção cirúrgica foi feita por Mechior em 1928. 3,4 Sabe-se que esse tumor ocorre de preferência em adultos do sexo feminino numa proporção de 4:1 com idade média de 54,4 anos (variando de 15 a 83 anos). ...
... 1,3,4 A primeira ressecção cirúrgica foi feita por Mechior em 1928. 3,4 Sabe-se que esse tumor ocorre de preferência em adultos do sexo feminino numa proporção de 4:1 com idade média de 54,4 anos (variando de 15 a 83 anos). 1,2,5 O quadro clínico do depende basicamente de sua localização e tamanho. ...
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O leiomiossarcoma de veia cava inferior é uma neoplasia rara originária das células do músculo liso da túnica média da veia cava inferior. Neste trabalho, relata-se o caso de um paciente do sexo feminino, 54 anos, com queixa inicial de dor abdominal em flanco direito há dois anos, associada a perda de peso de 7 kg em dois meses, inapetência e prostração, cuja ultrassonografia de abdome total evidenciou lesão sólida, hipoecogênica e de contornos levemente bocelados na adjacência da veia cava inferior e da cabeça pancreática. Foi realizada ressecção da lesão e envio do material para análise anatomopatológica, que evidenciou leiomiossarcoma de veia cava inferior. Essas lesões têm mau prognóstico e o diagnóstico precoce e ressecção completa com margens cirúrgicas livres é a única chance de sobrevida a longo prazo.
... Лейомиосаркома НПВ -медленно растущая опухоль, что нередко определяет позднюю диагностику. Наиболее постоянными симптомами являются боли в животе различной локализации, потеря массы тела, пальпируемое в животе объемное образование [16,18,19]. У части пациентов с поражением II-III сегментов НПВ (около 20 %) выявляются признаки синдрома Бадда -Киари [12,17]. ...
... С учетом, как правило, протяженного поражения НПВ и необходимости резекции значительного участка полой вены, актуальным является выбор метода реконструкции сосуда. На настоящий момент наиболее оправданным и часто выполняемым методом реконструкции является протезирование резецированного сегмента НПВ ПТФЭ-протезом [12,[14][15][16][17]19]. И если при резекциях I сегмента НПВ описываются варианты завершения оперативного пособия на полой вене перевязкой ее концов резекции, то при удалении опухолей II и III сегмента почти всегда есть необходимость реконструкции венозной магистрали. ...
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We report a case of intrapelvic locally recurrent rectal cancer after surgery in which clinical complete response was achieved after chemoradiotherapy with capecitabine and oxaliplatin (CAPOX), and discuss the relevant literature. The patient was a 67-year-old woman who had undergone lower anterior resection for Ra rectal cancer. Nine months after the surgery, contrast-enhanced CT scans of the abdomen and pelvis, which were taken because of perineal pain, revealed a 48-mm mass in the pelvic floor, and the patient was thus diagnosed as having local recurrence. Since the lesion was suspected to have invaded surrounding organs, radical resection was considered impractical, and chemoradiotherapy was chosen. Radiation (50 Gy) + eight courses of CAPOX were administered. A contrast-enhanced CT examination of the abdomen 5 months after starting chemoradiotherapy revealed a marked size reduction of the recurrent lesion, which was identified as a 23-mm soft tissue shadow, with no signs of distant metastasis. As of 9 years and 6 months after the diagnosis of intrapelvic local recurrence and 8 years after the completion of chemoradiotherapy, metastatic recurrence has not been reported.
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Leiomyosarcomas originating from the inferior vena cava are very rare malignant tumors with an extremely poor prognosis. We report FDG PET/CT findings of pathology-proven hepatic metastases from leiomyosarcoma originating from the inferior vena cava in a young woman whose initial presentation was worsening abdominal and chest pain.
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Primary osteogenic sarcoma of the breast is a rare clinical entity with few cases described in the literature. Unfortunately, the prognosis for these patients is poor when compared to invasive carcinomas of the breast. We report a case of a 58-year-old female who developed a primary osteogenic sarcoma of the breast five years after being treated for invasive carcinoma of the ipsilateral breast without the use of radiotherapy. 1. Introduction Breast lumps are the most common presenting complaint (40%) among patients presenting to general practice clinics with breast symptoms [1]. Among those referred to a tertiary centre, breast cancer is found to be the cause of the breast lump in 36% of patients [2]. Breast cancer is the most prevalent form of cancer observed in females globally, with 2.1 million persons affected each year. It accounts for the most cancer-related deaths among women with 627,000 dying from the disease in 2018 [3]. Carcinomas account for the majority of breast cancers with the contribution of sarcomas being less than 1% [4, 5]. Primary osteogenic sarcoma of the breast is particularly rare with published data being limited to case reports and small series [6]. When compared to breast carcinoma, primary osteogenic sarcoma has a sombre 5-year survival of 38% [6]. Given the rarity of this tumour and the reported poor outcomes of affected patients, it is critical that cases are reported in the literature to expand the available knowledge base in the hopes of eventually improving patient care. We report the case of a patient who developed a primary osteogenic sarcoma of the breast five years after being treated for invasive carcinoma of the ipsilateral breast. 2. Case Report A 58-year-old female presented with a 3 cm mass in the lower inner quadrant of the left breast. Ultrasound and mammography revealed a Breast Imaging Reporting and Data System [7] (BI-RADS) 4 lesion. Core biopsy of the lesion showed a grade 2 invasive ductal adenocarcinoma. The patient had wide local excision of the lesion along with axillary lymph node dissection. Pathological examination revealed a T2 N0 M0 grade 1 invasive ductal carcinoma with 14 examined lymph nodes showing no evidence of metastases. The patient subsequently refused adjuvant radiation and chemotherapy. Five years after her surgery, the patient presented with a painless, mobile, 15 cm mass involving the upper and lower outer quadrants of the left breast. No evidence of nipple retraction or discharge was observed. Mammography revealed a BI-RADS 5 lesion. A core biopsy showed breast tissue containing areas of bone formation with partially calcified osteoid material surrounded by stellate and spindle-shaped stromal cells. There was also osteoclast-like giant cells present. Contrast-enhanced computed tomography (CT) of the chest and abdomen revealed a large lobulated left breast mass with cystic and calcific foci, measuring with no evidence of invasion into the chest wall or skin. No pulmonary, hepatic, or bony lesions were identified. The patient refused mastectomy and chose to have a wide local excision of the breast mass (see Figure 1). (a)
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Aim: To evaluate the results of surgical treatment of patients with leiomyosarcoma of inferior vena cava. Material and methods: For the period 2003-2016 21 patients with leiomyosarcoma of IVC were operated at the abdominal department of Blokhin Russian Cancer Research Center. 23.8% patients (n=5) underwent longitudinal resection of IVC, 23.8% (n=5) - circular resection and ligation of IVC, 23.8% (n=5) - circular resection and ligation of IVC, left renal vein ligation, 9.5% (n=2) - circular resection and replacement of IVC, 9.5% (n=2) - circular resection and replacement of IVC, ligation of left renal vein, 9.5% (n=2) - circular resection and replacement of IVC, left renal vein implantation into the prosthesis. Results: The incidence of postoperative complications was 38% (n=8). Postoperative mortality was 4.8% (n=1). R1-resection was made in 4.8% patients (n=1). 1- and 2-year recurrence-free survival was 67% and 50% respectively. Overall 1- and 2-year survival was 86% and 68% respectively. Conclusion: In patients with leiomyosarcoma of inferior vena cava surgery is feasible and safe with satisfactory long-term results.
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In 1992 The International Registry of Inferior Vena Cava (IVC) Leiomyosarcomas was established to study the pathogenesis and natural history of the tumor and to support the most rational treatment. We collected 218 patients through a literature review and personal communications. We corresponded with several Authors to obtain up-to-date follow-up and any other data lacking at the initial review. The series was analyzed to identify predictive factors for clinical outcome. Tumors arose from the IVC lower segment in 80 patients, from the middle in 94 and from the upper in 41. A radical tumor resection was undertaken in 134 (61.5%) patients, 26 (11.9%) had a palliative resection and 58 (26.6%) were inoperable. An increased risk of death was associated with upper IVC segment involvement (p < 0.001), lower limb edema (p < 0.001), Budd-Chiari's syndrome (p < 0.001), intraluminal tumor growth (p < 0.001) and IVC occlusion (p < 0.001). Radical tumor resection was associated with better 5- and 10-year survival rates (49.4% and 29.5%). Tumors which arose from the middle segment fared better (56.7% and 47.3%) than those of the lower segment (37.8% and 14.2%) (p < 0.002). No palpable abdominal mass and abdominal pain were associated, in patients radically operated, with a better outcome and longer survival (p < 0.03 and p > 0.04 respectively). Despite the high rate of recurrence, radical tumor resection is the only long-term cure.
Article
Background and Objectives We operated on three patients with leiomyosarcoma of the inferior vena cava.Methods Complete excision was possible in all three patients.ResultsOne patient developed widespread metastasis at 23 months, one patient is alive with no evidence of disease at 70 months, and one patient is alive at 15 months. The third patient had subcutaneous and pulmonary metastases at the time of presentation, which are radiologically nondetectable at present following postoperative chemotherapy.Conclusions The clinicopathologic features, prognostic factors, and treatment of 130 cases found in a comprehensive literature search and our three cases are reported. J. Surg. Oncol. 1997;65:205–217. © 1997 Wiley-Liss, Inc.
Article
Leiomyosarcoma of the inferior vena cava is a rare and potentially curable tumor. Uncertainty about the results of treatment derives from lack of a large series in the same center and of a long-term follow-up of the published cases. A review of the world literature from 1871 to 1989 allowed us to collect information on 141 patients with inferior vena cava leiomyosarcoma to which our three cases have to be added. The tumor arose from the lower segment of the inferior vena cava (infrarenal portion) in 49 patients, from the middle segment (from the renal veins to the hepatic veins) in 59, and from the upper segment (from the hepatic veins to the right atrium) in 34 patients. Complete clinical, pathologic, and therapeutic data and up-to-date follow-up have been obtained through personal correspondence with several authors. All data have been examined with both univariate and multivariate analyses as predictive factors for outcome. Variables, associated with an increased risk of death from disease, included the involvement of inferior vena cava upper segment and a high-grade tumor. Patients who underwent a radical resection of the tumor (82 patients, 56.9%) had a significantly better survival (27.9% and 14.2%, 5- and 10-year survival rates, respectively). Of these patients, those with tumor of the inferior vena cava middle segment fared better than those with lower segment tumor (5- and 10-year survival rates were 48.3% and 34.4%, respectively, for middle segment tumor and 9.3% and 0.0% for lower segment tumor). Variables associated with a good outcome and longer survival were the presence of abdominal pain and the absence of a palpable abdominal mass. Despite the high rate of recurrence (52.4% of patients undergoing radical operation; median time, 25 months), radical resection of inferior vena cava leiomyosarcoma is the only chance for a long-term cure. An earlier and more accurate preoperative diagnosis, by means of modern diagnostic techniques (echography, CT scanning, magnetic resonance imaging) will allow a higher rate of radical resection to be performed with an increase in patient survival.
Article
We operated on three patients with leiomyosarcoma of the inferior vena cava. Complete excision was possible in all three patients. One patient developed widespread metastasis at 23 months, one patient is alive with no evidence of disease at 70 months, and one patient is alive at 15 months. The third patient had subcutaneous and pulmonary metastases at the time of presentation, which are radiologically nondetectable at present following postoperative chemotherapy. The clinicopathologic features, prognostic factors, and treatment of 130 cases found in a comprehensive literature search and our three cases are reported.
Article
Retroperitoneal leiomyosarcoma is a rare neoplasm for which complete surgical removal provides the only effective treatment, as local recurrence adversely affects prognosis. However, invasion of major vessels may occur, making complete resection difficult. This report describes the cases of three patients who required concomitant resection of parts of the inferior vena cava because of direct tumor invasion. The major vessels should be isolated in preference to the tumor capsule during surgery to prevent sudden exsanguination or incomplete tumor resection. Resection of a recurrent sarcoma or a solitary metastasis can be effective in selected patients.
Article
The inferior vena cava (IVC) is a rare site for primary soft tissue sarcoma. There are limited data in the literature regarding surgical management of the IVC and longterm survival of these patients. From 1982 to 2002, a total of 25 patients with primary IVC leiomyosarcoma was treated as inpatients and followed in a prospective database at Memorial Sloan-Kettering. Presenting symptoms, tumor characteristics, operative management, postoperative morbidity, and disease-specific survival were assessed for each patient. The 25 patients with primary IVC leiomyosarcoma accounted for 0.5% of all adult patients with soft tissue sarcoma treated during this time. The median patient age was 56 years (range 41 to 79 years). The three most common presenting symptoms were abdominal pain (52%), distention (20%), and deep venous thrombosis (12%). Of the patients, 21 (84%) underwent complete resection of the tumor. The IVC was managed in one of three ways: ligation (n = 11), primary/patch repair (n = 8), and expanded polytetrafluoroethylene tube grafting (n = 2). Among patients undergoing IVC ligation and primary/patch repair (n = 19), 11% had severe postoperative edema and none had worsening renal function. Local recurrence occurred in 33% of patients and distant recurrence occurred in 48% of patients. Patients undergoing complete resection had 3-year and 5-year disease-specific survival rates of 76% and 33%, respectively. There were no 3-year survivors among patients with incomplete resections. Complete resection of primary IVC leiomyosarcomas is feasible and associated with improved survival. The IVC can be managed by primary repair or ligation with a low risk of severe postoperative edema.
Diagnosis and management of tumours of the inferior vena cava
  • Tc Bower
  • A Stanson
Bower TC, Stanson A: Diagnosis and management of tumours of the inferior vena cava. In Vascular Surgery Edited by: Rutherford RB. Philadelphia: WB Saunders Company; 2000:2077-2092.
Diagnosis and management of tumours of the inferior vena cava. Vascular Surgery
  • T C Bower
  • A Stanson
  • TC Bower