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E-IJD CASE REPORT
Year : 2016  |  Volume : 61  |  Issue : 2  |  Page : 235
Radiation-induced vulvar angiokeratoma along with other late radiation toxicities after carcinoma cervix: A rare case report


Department of Radiation Oncology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India

Date of Web Publication1-Mar-2016

Correspondence Address:
Virendra Bhandari
401, Samyak Towers, 16/3 Old Palasia, Indore - 452 001, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.177791

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   Abstract 

Angiokeratoma including vulvar angiokeratoma is a very rare complication of radiation. Exact incidence is still unknown, we report a case that developed radiation-induced angiokeratoma of skin in the vulvar region along with other late radiation sequelae in the form of bone fracture, new bone formation, bone marrow widening, muscle hypertrophy, and subcutaneous fibrosis, 18 years after radiotherapy to the pelvic region for the treatment of carcinoma cervix. All these late radiation sequel are rare to be seen in a single patient, and none of the case reports could be found in the world literature.


Keywords: Late radiation toxicity, pelvis, vulvar angiokeratoma


How to cite this article:
Bhandari V, Naik A, Gupta K L, Kausar M. Radiation-induced vulvar angiokeratoma along with other late radiation toxicities after carcinoma cervix: A rare case report. Indian J Dermatol 2016;61:235

How to cite this URL:
Bhandari V, Naik A, Gupta K L, Kausar M. Radiation-induced vulvar angiokeratoma along with other late radiation toxicities after carcinoma cervix: A rare case report. Indian J Dermatol [serial online] 2016 [cited 2020 May 27];61:235. Available from: http://www.e-ijd.org/text.asp?2016/61/2/235/177791

What was known?
Radiation-induced angiokeratoma is known delayed effect and is seen very rarely.



   Introduction Top


Vulvar angiokeratoma occurs rarely angiokeratomas of the vulva are benign vascular lesions usually occurring in women during their reproductive age.[1] With reported incidence of 0.16% only.[2] On review of literature, only three cases of radiation-induced vulvar angiokeratoma could be found.[3],[4],[5] Here, we present a patient that developed radiation-induced vulvar angiokeratoma along with other late radiation toxicities 18 years after radiotherapy to the pelvis for cancer Cervix IIIB.


   Case Report Top


A 49-year-old female presented to with complaints of burning micturition associated with high-grade fever, difficulty in walking, and multiple skin lesions over the vulvar region in June 2011. At the age of 30 years, she was diagnosed as cancer Cervix IIIB for which she received external beam radiotherapy to pelvis on a telecobalt machine, 50 Gy/25#/37 days by two parallel opposed anteroposterior portals delivered on alternate days followed by low dose rate brachytherapy to a dose of 25 Gy to point A in September 1994. On completion of treatment, there was a complete regression of disease. The patient was asymptomatic and was on follow-up for past 16 years. In June 2011, the patient started having pain in pelvic area and difficulty in walking. X-ray pelvis revealed pathological fracture at right superior ischiopubic rami along with new bone formation lesion at inferior ischiopubic rami [Figure 1]. Contrast-enhanced computed tomography abdomen revealed the presence of subcentimeter para-aortic and bilateral inguinal lymph nodes with the presence of displaced fracture of right superior pubic ramus and also another old fracture at right inferior pubic ramus. The patient was conservatively managed. In the following month, she came with complaints of multiple, firm, red, warty lesion of varying size over external genitalia with episodes of leukorrhea [Figure 2]. There was no discharge from lesion. Since disease was locoregionally controlled, only symptomatic treatment was given.
Figure 1: Pathological fracture and new bone formation

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Figure 2: Subcutaneous fibrosis and multiple nodules over vulva

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In September 2012, she developed fever and severe low backache with progressive difficulty in walking. Magnetic resonance imaging revealed diffuse hyperintense signals in sacral ala and iliac wing on the left side more in subchondral marrow region with widening of ipsilateral sacroiliac joint space and enlargement of psoas muscle, ipsilateral iliacus, piriformis, and proximal adductor muscle were seen. A loculated cyst in the left iliacus muscle and fatty marrow changes in pelvic bones were also evident [Figure 3]a and [Figure 3]b. Vaginal wall thickening with extensive subcutaneous fibrosis along with multiple warty lesions with no evidence of disease at the primary site noted. Ultrasound-guided aspiration was done which ruled out tubercular infection. Positron emission tomography scan done in October 2012 revealed sclerotic lesions involving left sacroiliac joint (standardized uptake value 8.4) with increased joint space suggesting sacro-ileitis. Fluorodeoxyglucose (FDG) nonavid cortical break involving anterior pillar of right acetabulum, right inferior pubic ramus, and right sacral ala along with hypertrophy of left psoas, left iliacus, and piriformis muscle was noted, but no increased uptake was seen. No increased uptake was seen in vulvar lesions.
Figure 3: (a and b) Magnetic resonance imaging showing fatty marrow, diffuse hyperintense signals in sacral ala, and left iliac bone with widening of left sacroiliac joint and muscle changes

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Since the number and size of the warty lesions were increasing in size and were causing significant dyspareunia and psychological and emotional stress, we excised a small wart and sent for histopathology which revealed hyperplastic epidermis with focal hyperkeratosis. Papillary dermis showing numerous dilated vascular spaces, forming angiomatous mass [Figure 4]a and [Figure 4]b. Thus, a diagnosis of radiation-induced angiokeratoma was made. The patient was planned for wide excision of the lesions in view of above mentioned complains, but the patient refused for any further intervention and, therefore, she is kept on close follow-up. On last follow-up in December 2014, her pain has increased, and lesions over the vulvar region have increased in number and size.
Figures 4: (a and b) Hyperplastic epidermis with focal hyperkeratosis and papillary dermis showing numerous dilated vascular spaces forming angiomatous (a: ×10 and b: ×40)

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   Discussion Top


Angiokeratomas are benign lesions thought to be telangiectasias with secondary epithelial proliferation. Histologically, angiokeratomas are characterized by ectatic capillaries at the dermoepidermal junction, with reactive hyperkeratosis, acanthosis, and focal papillomatosis of the overlying epidermis.[6] Proposed mechanisms responsible for the development of angiokeratomas include primary degeneration of the vascular elastic tissue, sustained increased venous pressure causing the destruction of elastic fibers resulting in over distension, and chronic inflammation causing phlebectasia.[5]

Radiation-induced damage to blood vessels along with decreased blood return due to larger field size used in treatment could lead to an increase in venous blood pressure which in turn is known to cause angiokeratoma. Blood vessels are relatively resistant to radiation hence radiation-induced angiokeratoma is extremely rare.[5] Angiokeratomas are mostly asymptomatic lesions, and these increase in number and size slowly in radiated area.[7] Therapeutic intervention may be required if patient present with complains of dyspareunia, bleeding or pus discharge from some of the lesions, or associated pain and anxiety. In symptomatic cases, treatment modalities include surgical excision, electrocauterization, cryotherapy, or laser treatment, the choice of which largely depends on the size of the lesion.[8]

The present patient also developed progressively increasing skin lesions in the form of firm red, warty lesions over radiated area and started discharge later on along with leukorrhea. Similar lesions are seen in other patients reported in the literature.

Our patient also developed late radiation toxicities in the form of bone fractures, new bone formation, widening of joint space with fluid, fatty marrow, muscle hypertrophy, and subcutaneous fibrosis developing simultaneously along with vulvar angiokeratoma. Musculoskeletal complication of radiotherapy is often discovered incidentally during workup to check the presence of recurrence or metastatic disease. Routinely used radiation doses for the treatment for cancer cervix does not result in acute injury to muscle and bone. Radiation-induced changes in bones are usually detected years after radiation because of slow metabolic turnover of bones.[9] Radiation-induced changes in pelvis usually include osteopenia, increased bone density and widening and irregularity of the sacroiliac joints, and pelvic insufficiency fractures. In muscle, late changes are usually seen as discrete areas of fibrosis and muscle atrophy often with overlying volume deficits corresponding to the radiation treatment portals.[10]


   Conclusion Top


The presence of radiation-induced angiokeratoma in vulvar region and other late radiation toxicity in a single patient is very rare, and we could not find any such case in literature. The most likely cause for the development of these late effects in the present case was one field per day treatment using large anterior and posterior fields for the delivery of radiation in an overweight patient leading to increased integral dose.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Nomelini RS, Pansani PL, Guimarães PD, Martins-Filho A, Barcelos AC, Murta EF. Vulvar angiokeratoma. J Obstet Gynaecol 2010;30:418-9.  Back to cited text no. 1
    
2.
Naranjo Sintes R, Pereda Hernandez J, Delgado Florencio V, Linares Solano J. Angiokeratoma. Apropos of 93 cases. Med Cutan Ibero Lat Am 1988;16:255-61.  Back to cited text no. 2
    
3.
Wilkinson SJ, Stone IK. Atlas of Vulvar Disease. 2nd ed. Philadelphia PA: Lippincott Williams and Wilkins; 2008. p. 46.  Back to cited text no. 3
    
4.
Haidopoulos DA, Rodolakis AJ, Elsheikh AH, Papaspirou I, Diakomanolis E. Vulvar angiokeratoma following radical hysterectomy and radiotherapy. Acta Obstet Gynecol Scand 2002;81:466-7.  Back to cited text no. 4
    
5.
Smith BL, Chu P, Weinberg JM. Agiokeratomas of the vulva: Possible association with radiotherapy. Skinmed 2004;3:171-2.  Back to cited text no. 5
    
6.
Atherton DJ, Moss C. Naevi and other developmental defects. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 7th ed. Oxford: Blackwell Science; 2004. p. 151-14.  Back to cited text no. 6
    
7.
Cohen PR, Young AW Jr., Tovell HM. Angiokeratoma of the vulva: Diagnosis and review of the literature. Obstet Gynecol Surv 1989;44:339-46.  Back to cited text no. 7
    
8.
Rees R, Freeman A, Malone P, Garaffa G, Muneer A, Minhas S. Case study: The surgical management of angiokeratoma resulting from radiotherapy for penile cancer. ScientificWorldJournal 2009;9:339-42.  Back to cited text no. 8
    
9.
Howland WJ, Loeffler RK, Starchman DE, Johnson RG. Postirradiation atrophic changes of bone and related complications. Radiology 1975;117(3 Pt 1):677-85.  Back to cited text no. 9
    
10.
Lefaix JL, Delanian S, Leplat JJ, Tricaud Y, Martin M, Hoffschir D, et al. Radiation-induced cutaneo-muscular fibrosis (III): Major therapeutic efficacy of liposomal Cu/Zn superoxide dismutase. Bull Cancer 1993;80:799-807.  Back to cited text no. 10
    

What is new?
In this era when high energy photons and multiple fields are being used for the treatment of cancer, this rare late effect may be very rare.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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    Abstract
   Introduction
   Case Report
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    References
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