Downloaded from www.ajronline.org by 104.240.99.123 on 11/13/15 from IP address 104.240.99.123. Copyright ARRS. For personal use only; all rights reserved
543
Case Report
____________ 1 CT Appearance Lymphedema Ella Kazerooni1
and
of Angiosarcoma
Christian
Associated
with Chronic
Hessler1’2
Angiosarcoma is an uncommon, rapidly progressive neoplasm of the vascular endothelium that may be associated with chronic lymphostatic lymphedema of the extremities. Although it is seen more frequently in lymphedema of the upper extremities after mastectomy for breast carcinoma, it is also associated with idiopathic or congenital Iymphedema. This case illustrates the classic clinical course of angiosarcoma associated with idiopathic lymphedema and the previously undescribed CT appearance of lymphedema complicated by diffuse infiltrating angiosarcoma involving the entire right lower extremity. The extreme skin thickening, frequently nodular, with marked fibrous septal thickening and lakes of encapsulated fluid surrounding and directly involving muscle groups differs from the less marked honeycomb pattern typical of lymphedema on CT.
prevent rapid progression
of edema and discoloration
to the buttock
and lower back. Contrast-enhanced CT showed marked swelling of the leg manifested as increased attenuation of the subcutaneous fat, thickening
of the
of encapsulated
seen in typical
fibrous fluid
septa, in and
lymphedema.
and
around
a honeycomb muscles,
Extreme
pattern more
skin thickening
severe
below
with
lakes
than
that
the knee
was frequently nodular. An above-the-knee amputation was performed. Pathologic examination showed a diffuse infiltrating neoplasm that extended to the margins of resection. The malignant cells were arranged in solid sheets and surrounded well-defined vascular spaces, with some areas resembling a cavernous hemangiorna. Aggressive
angiosarcoma
skeletal
muscle
invasion.
After
was
present
in the dermis,
subcutis,
of the foot, leg, and thigh, with extensive surgery,
edema
and pain persisted
The patient died 2 weeks after surgery, could be undertaken. limb.
and
venous
in the remaining
before chemotherapy
Discussion Case
Report
A 26-year-old
woman
with a 16-year
history
of idiopathic
lymph-
edema of the right lower extremity was admitted for the evaluation of progressively increased swelling of the right leg associated with mottled discoloration of the skin. One year before, evaluation by the referring
physician
had revealed
lymphedema
confined
below
the
knee, which did not respond to conservative therapy. Mottling of the skin below the knee and significant local pain, thought to be cellulitis, developed
6 weeks
before
and heparin therapy, rapidly cm,
progressed
heparin,
and
admission.
mottling,
to the right groin. steroid
therapy
However,
erythema,
A 9-day
on the
despite
antibiotic
pain, and marked current
course
edema
of IV vancomy-
admission
failed
to
Received August 14, 1990; accepted after revision September 24, 1990. , Department of Radiology, University of Michigan Hospitals, 1 500 E. Medical 2 Present address: Route du Village 12, 1 066 Epalinges, Switzeriand. AJR 156:543-544,
March
1991 0361-803X/91/1
563-0543
© American
Roentgen
Angiosarcoma associated with chronic lymphedema of the upper extremity has been recognized as a complication in patients after mastectomy; more than 220 cases have been described in the literature [1 ]. This uncommon neoplasm occurs in 0.07-0.45% of patients who survive 5 or more years after radical mastectomy and occurs after an average latency of 10.25 years from mastectomy (range, 1 -26 years) [2]. This is much shorter than the duration of edema seen with the fewer reported cases of congenital or idiopathic lymphedema, 45.9 and 21 .8 years, respectively [3]. Angiosarcoma has been reported only in association with primary
Center
Dr.
,
Ann Arbor,
Ray Society
Ml 481 09-0030.
Address
reprint
requests
to E. Kazerooni.
Downloaded from www.ajronline.org by 104.240.99.123 on 11/13/15 from IP address 104.240.99.123. Copyright ARRS. For personal use only; all rights reserved
544
KAZEROONI
and secondary (lymphostatic) chronic edema, be it congenital, idiopathic, posttraumatic, or caused by filariasis, but never in renal, cardiac, or cirrhotic edema (high flow). The clinical course may be insidious, initially manifesting as increased edema or an innocuous skin discoloration, which progresses to confluent areas of ecchymosis, palpable nodules, and ulceration. In addition to local recurrence, pulmonary and pleural metastases are common [3]. Initially, angiosarcoma was thought to represent metastases from primary breast carcinoma [2]. Although it has also been speculated that postmastectomy radiation therapy could contribute to its occurrence, many postmastectomy patients with angiosarcoma have not received radiation therapy [3]. Neither of these causes plays a role in cases of idiopathic or congenital lymphedema. In all cases, chronic lymphostasis results from the absence or diminished number of normal regional lymph nodes or lack of functional afferent lymphatics, whether it is due to congenital hypoplasia, surgery, trauma, or infection. Proliferation of the lymphatic endothelium to improve collateral circulation has been hypothesized to occur in the setting of chronic lymphostasis, a stimulus that may predispose to the growth of mutant cells and malignancy [4]. Chronic stasis may slow the transport of fluid and protein from the interstitium into lymphatic tissue, altering the local response of the lymphatic system to antigens, as evidenced by the increased susceptibility of the lymphedematous extremity to infection. Just as there is an increased risk of cancer in systematically immunosuppressed visceral organ transplantation patients, localized immunosuppression is known to represent a site of preferential tumor growth, representing an “immunologically privileged site” [4]. Initially, these vascular sarcomas were presumed to arise from chronically dilated lymphatic vessels and were termed lymphangiosarcomas [2]. Histochemical and electron microscopic studies provide equal support for origin from the vascular endothelium, and Enzinger and Weiss [5] have stated that the distinction between lymphangiosarcoma and hemangiosarcoma is difficult and often arbitrary, despite microscopic, histochemical, and ultrastructumal evaluation, as both arise from endothelial cells. Lymphangiosarcoma and hemangiosarcoma together have been termed angiosarcoma [5]. Amputation or disarticulation has been successful in most series when performed as the initial primary therapy. Although such surgery is radical, such as intemscapulothomacic amputation or hip disarticulation, this appears to offer the best opportunity for long-term survival. Radical surgery at the time of recurrence does little to prevent widespread metastases. Radiation therapy and chemotherapy may have some role
AND
HESSLER
Fig. 1.-Contrast-enhanced raised nodules, thickened
AJR:156,
March
1991
CT scan shows marked skin thickening with fibrous septa, and lakes of encapsulated fluid.
when combined with radical surgery or in the treatment of metastatic disease [3]. The CT appearance of angiosarcoma has not been reported before. The increased attenuation of the subcutaneous fat, thickening of the fibrous septa, and a honeycomb pattern with lakes of encapsulated fluid seen in portions of the thigh have been described as the usual appearance of lymphedema [6]. However, the additional findings of extreme skin thickening, multiple cutaneous nodules, marked increased attenuation of the subcutaneous fat, and fluid collections both within and surrounding muscle groups, as seen in the leg, have not been described and correspond to the findings seen at amputation in this case (Fig. i ). Although the indications for CT evaluation in such cases are unlikely to arise frequently, clinically suspected deep venous thrombosis could not be evaluated otherwise, and the CT appearance is striking.
REFERENCES 1
.
2.
3. 4.
5. 6.
Martin MB, Kon ND, Kawamoto EH, Myers AT, Sterchi JM. Post-mastectomy angiosarcoma. Am Surg 1984:50:541-545 Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: report of six cases in elephantiasis chirurgica. Cancer 1948:1: 64-81 Sordillo PP. Chapman A, Hajdu SI, Magill GB, Golbey RB. Lymphangiosarcoma. Cancer 1981:48:1674-1679 Schreiber H, Barry FM, Russell WC, Macon WL, Ponsky JL, Pories WJ. Stewart-Treves syndrome: lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surg 1979:114:82-85 Enzinger FM, Weiss SW. Malignant vascular tumors. In: Soft-tissue tumors. St. Louis: Mosby, 1988:545-557 Hadjis NS, Can’ DH, Banks L, Pflug JJ. The role of CT in the diagnosis of primary lymphedema of the lower extremity. AJR 1985;144:361-364
543
Case Report
____________ 1 CT Appearance Lymphedema Ella Kazerooni1
and
of Angiosarcoma
Christian
Associated
with Chronic
Hessler1’2
Angiosarcoma is an uncommon, rapidly progressive neoplasm of the vascular endothelium that may be associated with chronic lymphostatic lymphedema of the extremities. Although it is seen more frequently in lymphedema of the upper extremities after mastectomy for breast carcinoma, it is also associated with idiopathic or congenital Iymphedema. This case illustrates the classic clinical course of angiosarcoma associated with idiopathic lymphedema and the previously undescribed CT appearance of lymphedema complicated by diffuse infiltrating angiosarcoma involving the entire right lower extremity. The extreme skin thickening, frequently nodular, with marked fibrous septal thickening and lakes of encapsulated fluid surrounding and directly involving muscle groups differs from the less marked honeycomb pattern typical of lymphedema on CT.
prevent rapid progression
of edema and discoloration
to the buttock
and lower back. Contrast-enhanced CT showed marked swelling of the leg manifested as increased attenuation of the subcutaneous fat, thickening
of the
of encapsulated
seen in typical
fibrous fluid
septa, in and
lymphedema.
and
around
a honeycomb muscles,
Extreme
pattern more
skin thickening
severe
below
with
lakes
than
that
the knee
was frequently nodular. An above-the-knee amputation was performed. Pathologic examination showed a diffuse infiltrating neoplasm that extended to the margins of resection. The malignant cells were arranged in solid sheets and surrounded well-defined vascular spaces, with some areas resembling a cavernous hemangiorna. Aggressive
angiosarcoma
skeletal
muscle
invasion.
After
was
present
in the dermis,
subcutis,
of the foot, leg, and thigh, with extensive surgery,
edema
and pain persisted
The patient died 2 weeks after surgery, could be undertaken. limb.
and
venous
in the remaining
before chemotherapy
Discussion Case
Report
A 26-year-old
woman
with a 16-year
history
of idiopathic
lymph-
edema of the right lower extremity was admitted for the evaluation of progressively increased swelling of the right leg associated with mottled discoloration of the skin. One year before, evaluation by the referring
physician
had revealed
lymphedema
confined
below
the
knee, which did not respond to conservative therapy. Mottling of the skin below the knee and significant local pain, thought to be cellulitis, developed
6 weeks
before
and heparin therapy, rapidly cm,
progressed
heparin,
and
admission.
mottling,
to the right groin. steroid
therapy
However,
erythema,
A 9-day
on the
despite
antibiotic
pain, and marked current
course
edema
of IV vancomy-
admission
failed
to
Received August 14, 1990; accepted after revision September 24, 1990. , Department of Radiology, University of Michigan Hospitals, 1 500 E. Medical 2 Present address: Route du Village 12, 1 066 Epalinges, Switzeriand. AJR 156:543-544,
March
1991 0361-803X/91/1
563-0543
© American
Roentgen
Angiosarcoma associated with chronic lymphedema of the upper extremity has been recognized as a complication in patients after mastectomy; more than 220 cases have been described in the literature [1 ]. This uncommon neoplasm occurs in 0.07-0.45% of patients who survive 5 or more years after radical mastectomy and occurs after an average latency of 10.25 years from mastectomy (range, 1 -26 years) [2]. This is much shorter than the duration of edema seen with the fewer reported cases of congenital or idiopathic lymphedema, 45.9 and 21 .8 years, respectively [3]. Angiosarcoma has been reported only in association with primary
Center
Dr.
,
Ann Arbor,
Ray Society
Ml 481 09-0030.
Address
reprint
requests
to E. Kazerooni.
Downloaded from www.ajronline.org by 104.240.99.123 on 11/13/15 from IP address 104.240.99.123. Copyright ARRS. For personal use only; all rights reserved
544
KAZEROONI
and secondary (lymphostatic) chronic edema, be it congenital, idiopathic, posttraumatic, or caused by filariasis, but never in renal, cardiac, or cirrhotic edema (high flow). The clinical course may be insidious, initially manifesting as increased edema or an innocuous skin discoloration, which progresses to confluent areas of ecchymosis, palpable nodules, and ulceration. In addition to local recurrence, pulmonary and pleural metastases are common [3]. Initially, angiosarcoma was thought to represent metastases from primary breast carcinoma [2]. Although it has also been speculated that postmastectomy radiation therapy could contribute to its occurrence, many postmastectomy patients with angiosarcoma have not received radiation therapy [3]. Neither of these causes plays a role in cases of idiopathic or congenital lymphedema. In all cases, chronic lymphostasis results from the absence or diminished number of normal regional lymph nodes or lack of functional afferent lymphatics, whether it is due to congenital hypoplasia, surgery, trauma, or infection. Proliferation of the lymphatic endothelium to improve collateral circulation has been hypothesized to occur in the setting of chronic lymphostasis, a stimulus that may predispose to the growth of mutant cells and malignancy [4]. Chronic stasis may slow the transport of fluid and protein from the interstitium into lymphatic tissue, altering the local response of the lymphatic system to antigens, as evidenced by the increased susceptibility of the lymphedematous extremity to infection. Just as there is an increased risk of cancer in systematically immunosuppressed visceral organ transplantation patients, localized immunosuppression is known to represent a site of preferential tumor growth, representing an “immunologically privileged site” [4]. Initially, these vascular sarcomas were presumed to arise from chronically dilated lymphatic vessels and were termed lymphangiosarcomas [2]. Histochemical and electron microscopic studies provide equal support for origin from the vascular endothelium, and Enzinger and Weiss [5] have stated that the distinction between lymphangiosarcoma and hemangiosarcoma is difficult and often arbitrary, despite microscopic, histochemical, and ultrastructumal evaluation, as both arise from endothelial cells. Lymphangiosarcoma and hemangiosarcoma together have been termed angiosarcoma [5]. Amputation or disarticulation has been successful in most series when performed as the initial primary therapy. Although such surgery is radical, such as intemscapulothomacic amputation or hip disarticulation, this appears to offer the best opportunity for long-term survival. Radical surgery at the time of recurrence does little to prevent widespread metastases. Radiation therapy and chemotherapy may have some role
AND
HESSLER
Fig. 1.-Contrast-enhanced raised nodules, thickened
AJR:156,
March
1991
CT scan shows marked skin thickening with fibrous septa, and lakes of encapsulated fluid.
when combined with radical surgery or in the treatment of metastatic disease [3]. The CT appearance of angiosarcoma has not been reported before. The increased attenuation of the subcutaneous fat, thickening of the fibrous septa, and a honeycomb pattern with lakes of encapsulated fluid seen in portions of the thigh have been described as the usual appearance of lymphedema [6]. However, the additional findings of extreme skin thickening, multiple cutaneous nodules, marked increased attenuation of the subcutaneous fat, and fluid collections both within and surrounding muscle groups, as seen in the leg, have not been described and correspond to the findings seen at amputation in this case (Fig. i ). Although the indications for CT evaluation in such cases are unlikely to arise frequently, clinically suspected deep venous thrombosis could not be evaluated otherwise, and the CT appearance is striking.
REFERENCES 1
.
2.
3. 4.
5. 6.
Martin MB, Kon ND, Kawamoto EH, Myers AT, Sterchi JM. Post-mastectomy angiosarcoma. Am Surg 1984:50:541-545 Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: report of six cases in elephantiasis chirurgica. Cancer 1948:1: 64-81 Sordillo PP. Chapman A, Hajdu SI, Magill GB, Golbey RB. Lymphangiosarcoma. Cancer 1981:48:1674-1679 Schreiber H, Barry FM, Russell WC, Macon WL, Ponsky JL, Pories WJ. Stewart-Treves syndrome: lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surg 1979:114:82-85 Enzinger FM, Weiss SW. Malignant vascular tumors. In: Soft-tissue tumors. St. Louis: Mosby, 1988:545-557 Hadjis NS, Can’ DH, Banks L, Pflug JJ. The role of CT in the diagnosis of primary lymphedema of the lower extremity. AJR 1985;144:361-364
