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Case
Report -
A 61-year-old man with progressive arm swelling
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Muhammad
A Amir, MD
A 61-year-old man admitted to the medical floor for progressive
swelling of his left upper extremity that started 1 week
before admission followed by pain in the same extremity.
He denied any aggravating or relieving factor for his complaints.
The patient admitted to a history of heroin and other illicit
drug abuse as well as heavy drinking. He also smoked 1 pack
per day for the last 45 years.
His
past medical history was significant for poly-substance
abuse, chronic pain syndrome, depression and post-traumatic
stress disorder. Family history was not contributory except
that his father died of a pancreatic carcinoma. The patient
was single and had never married. He had no allergies. His
current medications included methadone 10mg po QID, etodolac
500mg po BID, zolpidem 10mg po qHS, quetiapine 25mg po qHS.
Review
of systems at the time of initial assessment was remarkable
for decrease in apetite, lack of energy, generalized aches
and pain but no fever, cough, sputum shortness of breath,
chest pain or facial puffiness/swelling. Vital signs were
within normal limits. He was alert and oriented to time,
place, and person. Head and neck exam revealed no elevated
jugular venous pressure on the right but on the left there
was increased jugular venous pressure to 15cm, pupils were
equal and reactive and no nystagmus was noted. Heart exam
was normal and lungs were clear to auscultation bilaterally.
Abdominal and neurological exams were benign. In the examination
of the extremities the entire left arm was tight with non-pitting
edema with a diameter of 34cm versus a 30cm diameter of
the right arm with both measured at 15cm from the elbow.
Both arms had intact pulses and sensation. There were also
multiple injection (track) marks in both the upper and lower
extremities.
A
complete blood count showed an elevated white cell count
of 13.4 with 65 Segs, 1 band. The erythrocyte sedimentation
rate was 19. Hemoglobin and hematocrit were normal. Coagulation
and complete metabolic profiles were unremarkable.
The
patient was evaluated for deep venous thrombosis with negative
D-dimers and Doppler studies of the left upper extremity.
His chest roentgen showed a large left hilar and superior
mediastinal mass. A computed tomographic (CT) scan of the
thorax showed a homogeneous mass in the anterior mediastinum,
measuring 5.4 x 4.0 x 10.5 cm. This mass extended from the
thoracic inlet to the anterior-posterior window. A pulmonary
consultation was requested and he underwent a CT guided
fine needle aspiration biopsy of the mass in the thorax
for tissue diagnosis. The biopsy report showed small cell
carcinoma and he was referred to the oncology clinic where
he was started on combination chemotherapy after whole body
bone-scan and CT head did not show evidence of metastases.
Discussion
Superior
vena cava (SVC) syndrome, first described in 1757 by William
Hunter, results from the partial or complete obstruction
of the SVC. It occurs most often in men between the ages
of 50 and 70 years (61 in our case). The major signs and
symptoms of SVC include facial fullness or flushing, headache,
dyspnea, and cough. Less common complaints include edema
of the upper extremities, pain (as in our patient), dysphagia,
and syncope. Physical findings may include prominent distended
and tortuous venous systems in the face, neck and upper
trunk, papilledema, facial cyanosis and pleural effusion.
The
most common causes of this syndrome are extrinsic compression
of the superior vena cava and intraluminal venous thrombosis.
Up to 82% of cases result from obstruction caused by bronchogenic
carcinoma (the cause in our patient - small cell carcinoma).
Less common causes include granulomas (Histoplasmosis and
tuberculosis), lymphoma, and thrombosis of the SVC due to
intravascular foreign bodies (pacemaker leads, central venous
catheters).
Because
of the risks associated with SVC obstruction (central edema,
airway compromise), it is important to obtain a diagnosis
and institute therapy in a timely fashion. SVC obstruction
is sometimes suggested on plain radiographs as a widened
mediastinum. The use of contrast enhanced CT is much more
specific and can demonstrate collateral veins. Depending
on the clinical presentation, tissue diagnosis can be made
by bronchoscopy, fine needle aspiration biopsy (FNAB) or
mediastinotomy. The procedure of choice is based on the
clinical situation and risks. Caution is necessary when
performing venipuncture, lymph node biopsy, and bronchoscopy
because there may be profuse bleeding due to the high venous
pressures in the head and neck. In this patient, it was
decided to do FNAB after CT thorax to make tissue diagnosis
because of the increased risk of bleeding with bronchoscopy.
When
SVC syndrome is associated with malignancy, it is usually
fatal within 6-7 months without treatment. Overall survival
and symptom resolution depend on the ability to treat the
underlying disease. Symptom relief can be obtained through
simple measures such as supplemental oxygen, diuretics,
and steroids. Chemotherapy, radiation therapy or both can
effectively relieve the signs and symptoms when arising
from malignant disease. Surgical intervention is not recommended
except in selected individuals with fibrosing mediastinitis.
Chemotherapy
is the treatment of choice for sensitive tumors such as
small cell lung cancer and lymphoma. SVC syndrome does not
appear to be an independent prognostic factor, and its presence
should not be used to change the treatment approach. Rapid
initiation of chemotherapy can result in complete and partial
response rates of the SVC syndrome of more than 80% in small
cell lung cancer patients.
References
- Patel AM and SG Peters.
Clinical Manifestations of Lung Cancer. Mayo Clin
Proc. 1993;68: 273-277.
- DeCamp MM, SJ Swanson and DJ Sugarbaker.
The Mediastinum. Glenn's Thoracic and Cardiovascular
Surgery, 6th ed. ed by AE Baue, AS Geha, GL Hammond,
H Laks, and KS Naunheim. 1996: 658-659.
- Carr DT, PY Holoye, and WK Hong.
Bronchogenic Carcinoma. Textbook of Respiratory Medicine,
2nd ed. JF Murray and JA Nadel. 1994:1579-1580
- Wurschmidt F, Bunemann H, Heilmann
HP. Small cell lung cancer with and without superior
vena cava syndrome: a multivariate analysis of prognostic
factors in 408 cases. International Journal of Radiation
Oncology, Biology, Physics 33(1): 77-82, 1995.
- Dyet JF, Nicholson AA, Cook AM.
The use of the Wallstent endovascular prosthesis in
the treatment of malignant obstruction of the superior
vena cava. Clinical Radiology 48(6): 381-385,
1993.
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