Microbiology & parasitology

Parasites-cutaneous / subcutaneous / soft tissue

Filariasis



Topic Completed: 8 March 2021

Minor changes: 19 October 2021

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PubMed Search: Filariasis [title] breast

Andrii Puzyrenko, M.D., Ph.D.
Ziyan T. Salih, M.D.
Page views in 2020: 3,334
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Cite this page: Puzyrenko A, Jorns JM, Philip J, Salih Z. Filariasis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/breastfilariasis.html. Accessed October 21st, 2021.
Definition / general
  • Lymphatic filariasis is caused by a chronic mosquito borne parasitic infection that involves the lymphatic vessels and lymph nodes of a human host (Trends Parasitol 2017;33:83)
Essential features
  • Lymphatic filariasis is transmitted by mosquito bite
  • Infection is caused by the migration of filaria larvae into lymph nodes and lymphatic obstruction
Terminology
  • Elephantiasis
  • Bancroftian filariasis
ICD coding
  • ICD-10:
    • B74 - filariasis
      • B74.0 - filariasis due to Wuchereria bancrofti
      • B74.1 - filariasis due to Brugia malayi
      • B74.2 - filariasis due to Brugia timori
  • ICD-11:
    • 1F66 - filariasis
      • 1F66.2 - filariasis due to Brugia species
      • 1F66.3 - lymphatic filariasis
        • 1F66.30 - filariasis due to Wuchereria bancrofti
        • 1F66.31 - filariasis due to Brugia malayi
        • 1F66.32 - filariasis due to Brugia timori
        • 1F66.3Z - lymphatic filariasis, unspecified
Epidemiology
  • Lymphatic filariasis is a human parasitic infection (animal reservoirs are of minor epidemiologic importance or absent)
  • Lymphatic filariasis affects over 120 million people in 73 countries
  • Endemic in Central Africa, Southeast Asia, the Western Pacific, South America and the Caribbean (Nurs Clin North Am 2019;54:181)
  • In the USA, Charleston, South Carolina was the last known place with lymphatic filariasis; the infection was eradicated in the USA in the early 20th century (CDC: Lymphatic Filariasis [Accessed 18 October 2021])
  • M:F = 10:1 (Asian Pac J Trop Med 2011;4:581)
Sites
Pathophysiology
  • Bite from an infected mosquito introduces third stage infective filarial larvae onto the human skin
  • They penetrate into the bite wound to reach the lymphatics where they reside and develop into adults
    • Adult females give birth to numerous microfilariae (Asian Pac J Trop Med 2011;4:581)
    • Intact adult worms produce minimal tissue reaction but can cause lymphatic obstruction leading to lymphedema (peau d'orange skin - mimics malignancy)
    • Disrupted lymphatic drainage increases the susceptibility to repeated infections, most commonly streptococcal and fungal infections
    • Acute and chronic inflammation lead to fibrosis, hyperpigmentation and hyperkeratosis (Microcirculation 2013;20:349)
    • Degenerating / dying worms provoke an inflammatory reaction in the tissue, forming a mass with eosinophilic and granulomatous inflammation
  • Life cycle continues as microfilariae actively move through lymph and blood channels and are ingested by mosquitoes during a blood meal
    • After ingestion, the microfilariae develop into an infective larvae inside the mosquito through a 3 stage process
    • Third stage infective larvae migrate through the mosquito's proboscis and can infect another human when the mosquito takes a blood meal
Etiology
  • 3 species of nematode (roundworm) parasites cause lymphatic filariasis in humans (Trends Parasitol 2017;33:83):
    • Wuchereria bancrofti is the most common cause of infections worldwide (accounting for > 90% of cases)
    • Brugia malayi and Brugia timori can be the causative organisms in Asia
  • Infection is transmitted by mosquito vectors (Culex, Anopheles, Aedes and Mansonia species)
  • Humans are the definitive host
Diagrams / tables

Images hosted on other servers:
Life cycle of filarial worms

Life cycle of filarial worms

Missing Image

Diagnostic features

Clinical features
  • May be asymptomatic
  • Incubation period is variable and can be 8 - 16 months
  • Breast (local) (Asian Pac J Trop Med 2011;4:581)
    • Unilateral, nontender swelling, commonly in the upper outer quadrant
    • May be mobile from quadrant to quadrant and mimic fibroadenoma
    • May be filarial abscess
    • May be associated with enlarged lymph nodes and simulate breast carcinoma
    • Overlying skin has induration, hyperpigmentation, dilated veins
  • Systemic (filariatic fever) (Korean J Parasitol 2008;46:119)
    • Headache, fever, chills and general malaise
  • Vaginal bleeding is common; lymphedema is common at late stages
Diagnosis
  • Standard method to diagnose active infection is microscopic blood smear examination; sensitivity can be increased by concentration techniques
    • Peripheral blood smear (preferably taken after 8 p.m. and stained with either Giemsa or H&E stain) (Asian Pac J Trop Med 2011;4:581)
    • PCR (detection parasite antigen)
    • Serologic enzyme immunoassay test (detection of antifilarial IgG1 and IgG4)
    • Imaging with CT, MRI and ultrasonography (Filaria J 2003;2:3)
    • Histologic evaluation of surgically removed tissue
Laboratory
  • Antifilarial IgG1 and IgG4 antibody in peripheral blood by enzyme linked immunoassay (ELISA)
  • DNA sequencing and nucleic acid amplification by PCR
  • In chronic cases with lymphedema, lab tests are usually negative
  • Reference: CDC: Lymphatic Filariasis [Accessed 24 February 2021]
Radiology description
  • Real time ultrasound may show the movement of echogenic particles ("filarial dance") (Filaria J 2003;2:3)
Radiology images

Images hosted on other servers:
Unusual groupings of benign appearing calcifications

Unusual
groupings of
benign appearing
calcifications

Linear, tortuous, noncalcified opacities (right); serpiginous, linear and discontinuous calcifications (calcified worms, left)

Noncalcified opacities (left); calcified worms (right)

Caption

Linear, tortuous,
noncalcified
opacities

Oval shaped isodense lesion (arrow)

Oval shaped isodense lesion (arrow)

Cyst with few linear echogenic foci (arrow)

Cyst with few linear echogenic foci (arrow)

Dilated tubular channels; filarial worm (white arrows)

Dilated tubular
channels; filarial
worm (white arrows)

Prognostic factors
Case reports
Treatment
  • Medical treatments
    • Diethylcarbamazine (DEC) is recommended as potential monotherapy (Trends Parasitol 2017;33:83)
    • Single combined dose of ivermectin, albendazole and DEC resulted in clearance of microfilaria in 96% of affected patients for up to 3 years (currently recommended by the WHO as triple therapy) (Clin Infect Dis 2020;71:e68)
  • Surgical treatment may be an option (J Cutan Med Surg 2018;22:611)
  • Chronic management
    • Skin hygiene, regular washing with soap and water, using compressive bandages, cold / heat therapy, antibiotic and antifungal creams to prevent flares of lymphangitis (PLoS Negl Trop Dis 2015;9:e0004171)
Clinical images

Images hosted on other servers:
Right breast lump with pigmentation

Right breast lump with pigmentation

Lump in the right breast

Lump in the right breast

Resolved infection after DEC therapy

Resolved infection after DEC therapy

Gross description
Microscopic (histologic) description
  • Microfilariae are the diagnostic form
  • Intact adult filariae in lymph nodes are pathognomonic; may see dead filariae with surrounding granulomatous inflammation and calcifications (Clin Microbiol Rev 1998;11:366)
  • Eggs may be seen
  • Background shows increased eosinophils, neutrophils, plasma cells, macrophages and even granulomas with lymphatic vessel dilatation and fibrosis
  • Microfilarial morphology is better appreciated with a Giemsa stain
  • Presence or absence of a sheath and the pattern of nuclei in their tail are the main features used to distinguish the various species
    • Microfilariae of Wuchereria bancrofti are sheathed with no nuclei in the tip of the tail
Microscopic (histologic) images

Contributed by Bobbi Pritt, M.D. and AFIP images
Adult female worm Adult female worm Adult female worm Adult female worm

Adult female worm

Gravid female worm of<br><i>Wucheria bancrofti</i><br>surrounded by fibrosis

Gravid female worm of
Wucheria bancrofti
surrounded by fibrosis



Images hosted on other servers:
Missing Image Missing Image

Wuchereria bancrofti

Missing Image

Microfilaria

Cytology description
  • Microfilaria may appear as coiled structures with visible nuclei (Diagn Cytopathol 2011;39:8)
  • Fragments of adult worms may be present
Positive stains
  • Filaria are visible on Giemsa stain
Videos
Sample pathology report
  • Left breast, excisional biopsy:
    • Foreign body reaction to larval organisms showing a granulomatous inflammation with longitudinal calcified corpuscles, consistent with filariasis
Differential diagnosis
  • Granulomatous mastitis:
    • Shows well formed granulomas within lobules or adjacent to ducts
    • May show giant cells and chronic inflammation
    • Microabscesses may be present with lipid vacuoles
  • Bacterial or fungal lymphadenitis:
  • Cysticercosis:
    • Caused by larval cysts of the tapeworm Taenia solium
    • Larvae with similar stroma but contain a scolex, hooks and surrounding fluid filled "bladder"
    • Clinically presents with multiple lesions
  • Breast carcinoma:
    • Poorly defined palpable mass or area of thickening
    • Neoplastic epithelial proliferation with variable architecture, including glandular, cribriform, cords, trabeculae, papillae or solid growth
  • Fibroadenoma:
    • Painless, slowly growing, mobile, well defined, palpable mass
    • Calcifications may be present and appear as a cluster
    • Admixed benign glandular and stromal elements
  • Wuchereria bancrofti (1):
    • Sheath, no nuclei in the tip of the tail
  • Brugia malayi (2):
    • Sheath, 2 distinct nuclei in the tip of the tail
  • Loa loa (3):
    • Sheath, nuclei extending to the tip of the tail
  • Onchocerca volvulus (4):
    • No sheath, no nuclei in the tip of the tail
  • Mansonella perstans (5):
    • No sheath, nuclei extending to the tip of the tail
  • Mansonella ozzardi (6):
    • No sheath, no nuclei in the tip of the tail
  • Mansonella streptocerca (7):
    • No sheath, nuclei extending to the tip of the tail
Board review style question #1

A 37 year old woman presents with a nodule in her left breast associated with enlarged axillary lymph nodes. The nodule is surgically removed and shows the image above on review. What histological finding is most specific for lymphatic filariasis of the breast?

  1. Adult worm with eggs in lymph node
  2. Chronic lymphangitis with fibrosis
  3. Eosinophilic lymphadenitis
  4. Granulomatous lymphadenitis with calcifications
  5. Lymphatic vessel dilatation
Board review style answer #1
A. Adult worm with eggs in lymph node

Lymphatic filariasis of the breast due to Wuchereria and Brugia can show chronic lymphangitis with lymphatic vessel dilatation, eosinophilic lymphadenitis and granulomatous inflammation; however, these are not specific for filariasis and can be often seen due to other conditions. A key histologic feature supportive of filariasis is the presence of adult worms in lymph nodes.

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Reference: Filariasis
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