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| Journal of the New Zealand Medical Association,
02-June-2006, Vol 119 No 1235
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Cutaneous paraneoplastic syndrome (acrokeratosis
paraneoplastica) preceding squamous cell carcinoma of the glottic larynx
Gorkem Aksu, Ahmet Karadeniz
Paraneoplastic syndromes that occur in the minority of
cancer patients are the produced signs and symptoms at distant sites from the
tumour or its metastases. These syndromes may occur due to the production of
substances by tumoural lesions that directly or indirectly cause distant
symptoms or depletion of normal substances or host response to the tumours.
The best characterised paraneoplastic syndromes are those
producing ectopic hormones such as parathyroid hormone (PTH) or
adrenocorticotropin (ACTH)—in such cases the treatment of the underlying
malignancy leads to the disappearance of the hormone and the syndrome.1–3
A paraneoplastic syndrome may be the first sign of a
malignancy, so its recognition may be critical for early cancer detection. Also,
the secreted proteins that cause paraneoplastic syndromes can be used as tumour
markers during the therapy and follow-up for the evaluation of treatment
response or recurrence.2,3
Head and neck cancers have occasionally been reported in
association with paraneoplastic syndromes and to date there are only a few cases
in the literature about the presence of a paraneoplastic syndrome as the first
manifestation of a laryngeal cancer.1,4–6
In this report we present a case in which cutaneous
paraneoplastic syndromes preceded the histopathological diagnosis of glottic
laryngeal carcinoma, and we review the literature.
Case reportA 62-year-old man with a 35-pack per year smoking history
referred to dermatology department of our hospital for skin lesions developing 3
months previously. On physical examination, hyperkeratotic psoriasiform plaques
were present in both of his hands and soles. Both of the dorsal and palmar
aspects of the fingers and nail folds were involved and his toenails were also
thickened and dystrophic (Figure 1).
Figure 1. Hyperkeratotic psoriasiform plaques on the
left hand of our case
 Laboratory findings were within normal limits, and the only
abnormal finding in physical examination (except skin lesions) was significant
voice hoarseness. The patient claimed that skin lesions had preceded voice
hoarseness. With these findings, an endoscopic examination of larynx was
performed that showed a tumoural lesion in the left posterior ventricle
extending up to the vocal cord.
A biopsy was taken from the lesion and the histopathological
diagnosis was squamous cell carcinoma of the larynx. Computed tomography (CT)
showed that the lesion was limited to the glottic region and there was no
lymphadenopaties in the neck. CT of chest also demonstrated no pathology.
The patient was diagnosed as having glottic larynx cancer
and acrokeratosis paraneoplastica, and he was treated with 66 Gy external
radiotherapy with 2 Gy daily fractions. The skin lesions (except the nail
dystrophy) significantly resolved after the completion of radiotherapy, and the
patient is still alive 3 years later with no evidence of disease.
DiscussionA wide variety of cutaneous syndromes are associated with
malignancies. These syndromes may precede, follow, or be concurrent with the
underlying malignancy. The most critical point is that once a potential
cutaneous paraneoplastic syndrome has been diagnosed, an extensive systemic
evaluation emphasising the malignancies most strongly associated with that type
skin lesion shall be undertaken.
The physicians especially should be aware of some cutaneous
lesions that are uncommon and usually associated with cancer.1,2 Acrokeratosis
paraneoplastica is a typical example of such lesions since it is one of the most
rare cutaneous paraneoplastic syndromes and is typically present in patients
with squamous cell carcinoma of the oesophagus, head, and neck or lungs. The
eruptive lesions are characteristically hyperkeratosic, resembling psoriasis and
favouring acral sites and nails.
In most of cases, including ours, acrokeratosis
paraneoplastica is the first sign of the underlying occult malignancy. Antigenic
cross-reaction of basement membrane and tumour antigens, and the secretion of
some growth factors such as insulin-like growth factor-1 (ILGF-1) or
transforming growth factor-alpha (TGF-alpha), are thought to be possible
mechanisms causing this syndrome—as squamous cell carcinomas have been
shown to synthesise and secrete these autoimmune growth factors.6–9
Head and neck squamous cell carcinomas are the most
frequently associated malignancy with this syndrome, and oropharynx or larynx
cancers make up more than 60% of the cases reported in the literature.8,9 Bazex
described three stages for cutaneous lesions of acrokeratosis paraneoplastica;
he reported that fingers, toes, soles, and (in some cases) the ear helices and
nose are affected usually in a symmetrical fashion.
Bazex also mentioned that the average time between the
appearance of the cutaneous lesions and the detection of the underlying tumour
is about 11 months, but this interval is shorter in some of the reported cases
in the literature, including our case.10 The skin eruptions may resolve with the
treatment of underlying malignancy but in some cases the lesions persist or
reappear with the recurrence of the tumour. UV-A phototherapy or retinoids and
oral psoralen have been used in some cases with the reportage of limited
benefits.7–9
Seborrheic keratosis which is generally seen in elderly
patients can also appear as a paraneoplastic cutaneous syndrome. The
Leser-Trelat sign which is characterised by the sudden appearance and rapid
increase in the number and size of seborrheic keratoses is important since these
lesions can be associated with internal malignancies. The most common malignancy
is adenocarcinoma of the stomach but the syndrome can also be present in
patients with breast cancer as well as squamous cell carcinomas of head, neck,
and lung.8,10
As described above, early diagnoses of these lesions is
critical because, with our case, these cutaneous lesions may precede or be
concurrent with an underlying malignancy. Therefore paying careful attention to
such lesions may lead earlier detection of at least some malignancies.
Author information:
Gorkem Aksu, Assistant Professor, Faculty of Medicine, Radiation Oncology
Department, Kocaeli University, Kocaeli; Ahmet Karadeniz, Professor, Radiation
Oncology Department, Oncology Institute, Istanbul University; Turkey
Correspondence:
Gorkem Aksu, Yahyakaptan Mahallesi, F 29 Blok, Daire: 12, Yahyakaptan, Kocaeli,
Turkey. Fax: +90 262 3038003; email: aksugorkem@yahoo.com
References:
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