(Chest. 2001;120:1725-1727.)
© 2001
American College of Chest Physicians
A Cystic Upper Lobe Lesion in a Healthy Nonsmoking Man*
Colm T. Leonard, MD;
Ann Weinacker, MD, FCCP;
Gerald Berry, MD and
Richard I. Whyte, MD, FCCP
*
From the Division of Pulmonary and Critical Care Medicine (Drs. Leonard and Weinacker) and the Departments of Histopathology (Dr. Berry) and Cardiothoracic Surgery (Dr. Whyte), Stanford University Medical Center, Stanford, CA.
Correspondence to: Colm T. Leonard, MD, North West Lung Centre, Wythenshawe Hospital, Southmoor Rd, Manchester M23 9LT, United Kingdom; e-mail: colmleonard{at}yahoo.com
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Introduction
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A 57
-year-old man presented with cough, purulent blood-streaked mucus, and
sinus congestion, which resolved with a course of
amoxicillin/clavulanate therapy. The patient was noted to have a left
upper lobe cystic lesion on a chest radiograph (Fig 1
) and a CT scan (Fig 2
). Sputum cytology testing results were negative. Sputum cultures
initially revealed Mycobacterium gordonae, but this did not
grow on subsequent serial sputum analyses and was presumed to be a
contaminant. The patient was a lifelong nonsmoker, was feeling well,
had no weight loss, fevers, or sweats, and had not experienced any
unusual environmental exposures. The only unusual features in his
medical history were a cholecystectomy and a skin lesion on his scalp,
which had been removed 3 years previously.
The patient had no recurrence of symptoms and no change in the size of
the lesion as assessed by CT scan over the following 7 months, and so
he declined further intervention. However, 11 months after the initial
presentation, the hemoptysis recurred and persisted despite treatment
with broad-spectrum antibiotics. At that time, the lesion looked larger
on a chest radiograph and a CT scan. The patient still felt well, apart
from the hemoptysis, and sputum culture findings were negative for
bacterial, fungal, or mycobacterial organisms.
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What is the diagnosis?
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Diagnosis: Angiosarcoma of the left upper lobe, presumed metastatic
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Bronchoscopy revealed blood oozing from the left upper lobe
bronchus and abnormal mucosa distally. Bronchoscopic biopsies revealed
angiosarcoma with identical histology to that of the scalp lesion that
had been removed 3 years previously and treated with local
radiotherapy. Immunohistochemistry demonstrated reactivity with the
endothelial markers CD34 and CD31. The patient underwent a left upper
lobectomy (histology of resected specimen in Fig 3
) with an uneventful postoperative course; 16 months after his
lobectomy, there is no evidence of thoracic recurrence of his
angiosarcoma as shown by chest radiograph or CT scan. Fifteen months
after undergoing the lobectomy, the patient underwent a barium small
bowel follow-through for left lower quadrant abdominal pain that had
been present for several months. Nothing had been found on
esophagogastroduodenoscopy, colonoscopy, and abdominal CT scan 4 to 5
months previously. On the barium examination, an 8- to 9-cm mass-like
lesion was found in a loop of small bowel overlying the left lower
quadrant, which was confirmed by repeat CT scan. The patient underwent
resection of this lesion, which was an angiosarcoma, and another small
bowel angiosarcoma lesion, which had not been seen on any of the
radiographic studies, was found during the laparotomy and was also
removed. It is now approximately 5 years since this patient’s original
scalp angiosarcoma was removed.
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Figure 3.. Angiosarcoma composed of endothelial-lined
papillae expanding the submucosal bronchial tissues (hematoxylin-eosin,
original x100). Insert: high-power magnification
showing plump epithelioid endothelial cells with irregular
hyperchromatic nuclei and division figures (hematoxylin-eosin, original
x400).
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Discussion
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Cases of scalp angiosarcoma metastasizing to the lung have been
described previously.1
2
3
4
5
6
7
8
9
10
11
12
This case is unusual as
angiosarcomatous pulmonary metastases in past reports usually have been
multiple, the patients have been persistently symptomatic, and the
disease has progressed rapidly.
Clinical Features
Angiosarcomas are rare malignant tumors of vascular origin,
accounting for 2 to 3% of all sarcomas, and seem to have a
predilection for the scalp.1
Angiosarcomas, regardless of
their source, are particularly liable to metastasize to the
lung.2
3
A 1993 review2
stated that primary
angiosarcomas of the lung are extremely rare, with only eight cases
having been reported in the literature at that time.
Angiosarcomas are especially common in the sixth through seventh decade
of life, are more common in men, and show a particular predilection for
the head and neck.8
Patel and Ryu2
reported 15 cases of pulmonary angiosarcoma
from the Mayo Clinic. The majority (12 of 15 patients) had symptoms
ranging from weight loss to cough, hemoptysis, chest pain, dyspnea, or
fever. Ten of 15 patients had no physical signs. The sources of the
pulmonary lesions varied as follows: heart (three patients); breast
(three patients); forearm (one patient); scalp (one patient); skull
(one patient); liver (one patient); tibia (three patients); jugular
vein (one patient); and chest wall (one patient). One case failed to
reveal a primary source of disease for the patient. The patient with a
primary scalp angiosarcoma had a pneumothorax and bilateral pulmonary
nodules in addition to hemoptysis. Metastases to the lung occur
in 60 to 80% of cases of cutaneous and cardiac
angiosarcomas.2
Cavitation, pneumothorax, or hemothorax
have been reported to be more common with angiosarcomas originating
from the scalp. In a 1987 review of 95 patients with angiosarcoma,
those patients with scalp lesions (33 of 95 patients) had a much higher
incidence of metastatic pulmonary complications (ie,
pneumothorax or hemothorax). All patients with pulmonary involvement in
this series had multiple pulmonary lesions.3
Radiology
There are a variety of radiographic appearances associated with
metastatic pulmonary angiosarcoma. In the series of Patel and
Ryu,2
11 patients had multiple nodules, 3 patients had
linear infiltrates, 2 patients had pleural effusions, and 1 patient
each had a diffuse alveolar pattern, pneumothorax, and normal
chest radiograph. A distinctive appearance of some
angiosarcomatous pulmonary metastases as thin-walled, cystic, annular
lesions also has been reported in association with spontaneous
hemopneumothorax.4
Miller et al9
reported a case of a 57-year-old man with a
2-month history of malaise, low-grade fever, weight loss, hemoptysis,
and spontaneous pneumothorax. A chest radiograph and a CT scan revealed
multiple delicate, thin-walled, annular lesions in both lung fields.
The results of a CT-guided needle biopsy confirmed a diagnosis of
angiosarcoma. On a subsequent physical examination, a scalp lesion was
discovered that proved to be an angiosarcoma.
The mere appearance of cystic or bullous lung changes on CT scans in
patients with known sarcomas has been proposed as a sign heralding
metastatic disease.5
Primack et al10
reported 12 patients with hemorrhagic
pulmonary nodules showing central soft tissue attenuation with
surrounding ground-glass attenuation. Infectious causes accounted for 8
of 12 cases (invasive aspergillosis, 3 cases; candidiasis, 2 cases;
cytomegalovirus, 1 case; herpes simplex virus, 1 case; and
coccidioidomycosis, 1 case). Noninfectious causes accounted for
4 of 12 cases (Wegener’s granulomatosis, 2 cases; metastatic
angiosarcoma, 1 case; and Kaposi’s sarcoma, 1 case).
Pathology
The diagnosis of pulmonary angiosarcoma (Fig 3)
, either
primary or metastatic, can be problematic.13
Because the
tumors are often hemorrhagic, symptoms may be misinterpreted as a
pulmonary hemorrhage, hemorrhagic bronchopneumonia, or diffuse alveolar
hemorrhage. Clinical history and histologic clues are helpful.
These include irregular anastomosing networks lined by enlarged
hyperchromatic cells. Cytologic atypia and division figures distinguish
angiosarcoma from reactive vascular proliferations. In some cases of
metastatic angiosarcoma, the tumor is centered around native pulmonary
vessels without a host inflammatory response. Immunohistochemical
studies are useful for the poorly differentiated or epithelioid
variants.
Treatment
These tumors have not been shown to be
chemosensitive.2
Radiotherapy has been used, but usually
it is followed by local recurrence and metastasis.6
We can
find no previous report of the resection of an isolated metastatic
pulmonary angiosarcoma.
Prognosis
There is no prospective trial of survival in patients with
metastatic angiosarcomas, but the survival rate of patients reported in
the literature has been uniformly poor. Even with combined-modality
therapy, the prognosis is generally poor, with a 5-year survival
rate of 5 to 10%.6
Local recurrence and metastasis
usually occur within 2 years.7
The most common sites of
metastasis are regional lymph nodes and the lung.
In the series reported by Patel and Ryu,2
the clinical
course was dismal. Ten of 11 patients with an antemortem diagnosis died
an average of 9 months after the recognition of metastatic lung
involvement.
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Summary
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A cystic lung lesion in a patient with a medical history that
includes a scalp lesion should raise the suspicion of metastatic
angiosarcoma. However, as evidenced by our case, not all metastatic
angiosarcomas exhibit the multiple pulmonary lesions and rapid disease
progression of cases previously reported in the literature.
Received for publication November 29, 1999.
Accepted for publication November 16, 2000.
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References
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Lawrence, W, Jr, Donegan, WL, Natarajan, N, et al (1987) Adult soft tissue sarcomas. Ann Surg 205,349-359[ISI][Medline]
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Patel, AM, Ryu, JH (1993) Angiosarcoma in the lung. Chest 103,1531-1535[Abstract/Free Full Text]
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Kitagawa, M, Tanaka, I, Takemura, T, et al (1987) Angiosarcoma of the scalp: report of two cases with fatal pulmonary complications and a review of Japanese autopsy registry data. Virchows Arch 412,83-87[CrossRef]
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Cardoza, DW, Phillips, LC, Chen, I, et al (1966) Cystic pulmonary metastasis complicating angiosarcoma of the scalp. Calif Med 105,210-214
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Sarno, RC, Carter, BL (1985) Bullous change by CT heralding metastatic sarcoma. Comput Radiol 9,115-120[CrossRef][ISI][Medline]
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Visor, RL, Sheridan, MF, Burgess, LPA (1997) Angiosarcoma of the scalp. Otolaryngol Head Neck Surg 117,138[CrossRef][ISI][Medline]
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Rufus, JM, Tran, LM, Sercarz, J, et al (1993) Angiosarcoma of the head and neck, the UCLA experience 1955–1990. Arch Otolaryngol Head Neck Surg 119,973-978
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Batsakis, JG, Rice, DH (1981) The pathology of head and neck tumors: vasoformative tumors; part 9B. Head Neck Surg 3,326-339[ISI][Medline]
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Miller, SR, Chua, GT, Jay, SJ (1993) General case of the day: angiosarcomatous pulmonary metastases. Radiographics 13,1153-1155[Free Full Text]
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Primack, SL, Hartman, TE, Lee, KS, et al (1994) Pulmonary nodules and the CT halo sign. Radiology 190,513-515[Abstract/Free Full Text]
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Naka, N, Ohsawa, M, Tomita, Y, et al (1995) Angiosarcoma in Japan: a review of 99 cases. Cancer 75,989-996[CrossRef][ISI][Medline]
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Sheppard, MN, Hansell, DM, Du Bois, RM, et al (1997) Primary epithelioid angiosarcoma of the lung presenting as pulmonary hemorrhage. Hum Pathol 28,383-385[CrossRef][ISI][Medline]
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Colby, TV (1995) Malignancies in the lung and pleura mimicking benign processes. Semin Diagn Pathol 12,30-44[ISI][Medline]
TOP
Introduction
What is the diagnosis?
