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patient was lost to follow-up but and presented 1 year In one case there was described the use of wide local
later, again with a firm nodule at the right inguinal region excision with adjuvant radiotherapy for malignant eccrine
measuring 1 cm × 1 cm. Repeat CT of chest/abdomen/ acrospiroma of the scalp and left parotid, which eventually
pelvis revealed a soft tissue thickening with a solitary, had local recurrence in the parotid region after 2 years. In
[3]
round lesion in the right inguinal region along with an another case there was described a more radical surgical
expansile soft tissue density lesion with bone erosion approach of amputation of the leg with regional lymph
involving the left 9th rib, suggestive of metastases node dissection. This was required for clinical control
[Figure 4]. of extremity acrospiroma. In another reported case, a
[10]
66-year-old female with a recurrent malignant acrospiroma
Patient was again subjected to wide local excision of right of the chest treated by wide radical resection, including
inguinal lesion and also of 9th rib mass. Three nodes also chest wall excision, followed by reconstructive surgery
were dissected, with the largest measuring 3 cm × 3 cm, and radiotherapy. After 16 months, there was no evidence
along with a 4 cm × 5 cm mass present over and adherent of local recurrence or distant metastasis. [11]
to 9th rib postero-laterally. Microscopy revealed sections
from both the rib lesions as well as groin nodes showing One group described the role of radiotherapy in malignant
infiltration by malignant sweat gland tumor. Marrow of eccrine acrospiroma, wherein 3 cases of malignant
rib bone revealed infiltration of same tumor. Bilateral acrospiroma were treated with postoperative radiotherapy
iliac bone marrow aspiration and biopsy were negative with doses of 71-76 Gy to the primary surgical bed and
for tumor. 50 Gy to the draining lymph node basin, with modest
disease-free survival (27 and 35 months) in 2 of the 3 cases.
Patient was subsequently given adjuvant chemotherapy They suggested that certain histological features such as
consisting of paclitaxel 175 mg/m and cisplatin 80 mg/m dermal lymphatic invasion, nerve sheath involvement,
2
2
every 3 weeks for 6 cycles. The patient is on regular follow deep structural infiltration, positive resected margins, and
up and in clinical remission for the past 18 months. extracapsular lymph node extension may identify a high risk
of recurrence and merit postoperative radiotherapy. The
[12]
DISCUSSION role of chemotherapy in eccrine sweat gland carcinomas,
and especially malignant acrospiromas, is not clear. Various
Acrospiromas are cutaneous tumors of sweat duct origin case reports and case series have reported on the use of a
and differentiation. They usually present as slowly multitude of drugs in various sweat gland carcinomas
enlarging 1 cm to 2 cm nodules in middle-aged or including cyclophosphamide and doxorubicin, bleomycin,
older adults without site predilection. The term eccrine cisplatin, mitomycin C, with partial response and a median
acrospiroma was first coined by Johnson and Hewig, in duration of response of 4 to 16 months. [13-16] There are
1969, because, by histologic and histochemical studies, also isolated reports of response to taxanes (docetaxel and
the cells were believed to mimic those of the eccrine sweat paclitaxel). [17]
gland. Histologically, these lesions are subclassified
[4]
according to the location of the tumor in relation to the Analyzing all the available literature, we conclude that
epidermis, with those confined primarily to epidermis as wide local excision is the treatment of choice for these rare
epidermal acrospiroma and those involving both epidermis skin appendage tumors when localized, while adjuvant
and dermis as juxtaepidermal acrospiroma or just eccrine radiotherapy may provide some additional benefit in local
poroma. Those which are confined exclusively to dermis control. Poly-chemotherapy is thought to be an option for
or have minimal connection to epidermis are terme dermal more extensive lesions and paclitaxel-containing regimens
acrospiroma or hidradenoma. [1] could provide a viable option for palliation. However,
more evidence in the form of case series and case reports
Malignant acrospiroma comprises a group of rare is needed to establish its usefulness.
epidermal, juxtaepidermal, and dermal ductal carcinomas
occurring over the head and neck, anterior trunk, or Financial support and sponsorship
extremities. [5,6] One series described an incidence of only Nil.
five cases in a group of 750,000 evaluated individuals over
an eight-year period. They follow a predictable pattern Conflicts of interest
[7]
from the initial tumor site to regional lymph node and There are no conflicts of interest.
ultimately to systemic spread. [3,8]
REFERENCES
In the present case, the lesion recurred multiple
times despite initial wide local excision and adjuvant 1. Fletcher CD. Diagnostic histopathology of tumors. 3rd ed.
radiotherapy, carried out following the first recurrence. Philadelphia: Churchill Livingstone; 2000. p.1457-8.
Secondly, the lesions were slowly growing with delayed 2. Obaidat NA, Alsaad KO, Ghazarian D. Skin adnexal neoplasms --
recurrent nodal and bone metastases and hence the need part 2: an approach to tumours of cutaneous sweat glands. J Clin
Pathol 2007;60:45-159.
for prolonged follow up. 3. Holden B, Colome-Grimmer M, Savage C, Stierman K, Pou AM.
Malignant eccrine acrospiroma with metastases to the parotid. Ear
Malignant acrospiromas are treated by wide local Nose Throat J 2002;81:352-5.
excision, but with a local recurrence rate of around 50%. 4. Johnson BL Jr, Helwig EB. Eccine acrospiroma. A clinic-pathologic
[9]
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