Kikuchi-Fujimoto Disease Masquerading as Metastatic Papillary

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Transcript Kikuchi-Fujimoto Disease Masquerading as Metastatic Papillary

Kikuchi-Fujimoto Disease Masquerading as Metastatic
Papillary Carcinoma of the Thyroid
Manuel Villa,
MD ,
Shailesh Garg,
MD ,
Thomas Mathew,
MD ,
Louis-Joseph Auguste, FACS,
Northshore-Long Island Jewish Health System, Manhasset, New
Case Presentation
Kikuchi-Fujimoto disease also known as histiocytic necrotizing
lymphadenitis is a rare cervical inflammatory lymphadenitis, most
commonly seen in young Asian women, although it might be associated
with autoimmune diseases and commonly follows a self-limited course.
We present an unusual case of Kikuchi-Fujimoto disease masquerading
as metastatic papillary carcinoma of the thyroid.
A 30-year-old young female presented 2 months post-partum with
complaints of neck pain and fever with CT scan showing enlarged rightsided lymph nodes with a thyroid nodule. A subsequent biopsy of the
thyroid nodule showed papillary thyroid carcinoma and reactive
inflammation of the lymph node. She was electively taken for surgery
when a total thyroidectomy, central node dissection and a right modified
lymph node dissection was performed for enlarged lymph nodes. After
an uneventful recovery, pathology came back as papillary carcinoma of
thyroid with one metastatic lymphadenopathy and several other lymph
nodes with histiocytic necrotizing lymphadenitis.
This co-existence of Kikuchi-Fujimoto disease with metastatic
papillary thyroid cancer at presentation is unusual and presents a
challenging and complex management dilemma.
With no significant past medical history of SLE, tuberculosis,
lymphoma or autoimmune disorder, her presumptive diagnosis was
thyroid cancer with possible metastasis with coexisting viral infection.
She underwent thyroid nodule FNA and lymph node biopsy that
showed papillary thyroid cancer and reactive inflammatory changes in
the lymph node. Subsequently, she underwent a total thyroidectomy with
central node dissection and a right radical modified lymph node
dissection that proceeded uneventfully. During her surgery she had
extensive lymphadenopathy that resulted in a type III comprehensive
neck dissection encompassing submandibular gland and lymph nodes
level I to VI. Pathology showed papillary carcinoma of thyroid with one
positive lymph node and remaining lymph nodes showing necrotizing
histiocytic lymphadenitis. Patient recovered well from her surgery and
underwent radioactive iodine I-131 treatment postoperatively with post
therapy whole body I-131 scan demonstrating no iodine avid tissue in
the thyroid bed.
Submandibular Space
Kikuchi-Fujimoto disease is a histiocytic necrotizing lymphadenitis
which is a rare and benign condition that has been mainly described in
women younger than 40 years of age. It has been described in men too
and practically all ethnic groups though more commonly in Asian
people. It can mimic other diseases such as lymphoma, tuberculous
adenitis, metastatic disease, SLE, cat scratch disease and infectious
mononucleosis. The pathogenesis is unclear but is believed to be an
immune response of T cells and histiocytes to an unknown inciting
agent such as EBV, HHV 6 & 8, HIV, toxoplasma and paromyxoma
viruses. Cellular destruction is hypothesized to be due to apoptotic cell
death mediated by CD8 T lymphocytes.
It usually presents with fever, fatigue, weight loss, painful cervical
lymphadenopathy, arthritis and sometimes rash. Lymphadenopathy is
usually cervical but may involve axillary, mediastinal, and iliac nodes.
Anemia, leukopenia, and elevated ESR can be seen on laboratory studies.
Lymph node biopsy needs to be done to confirm the disease & to
exclude other serious disorders like metastatic disease and lymphoma.
Pathology usually shows necrotic foci on gross examination and
paracortical foci with histiocytic infiltrate on microscopic examination.
Although uncommon, a recurrence rate of 3-7% has been reported.
Signs and symptoms usually resolve in 2 to 6 months and there is no
effective treatment though high dose glucocorticoids with intravenous
immunoglobulin have been shown to be of some benefit.
The coexistence of Kikuchi disease and papillary thyroid cancer in
this patient presented a complex and challenging clinical scenario
especially, the decision to perform a neck dissection for clinically positive
nodes despite FNA showing reactive changes and the intraoperative
decision regarding how extensive should the node dissection be. In
summary, although Kikuchi’s disease is a rare entity, we should consider
it in the differential diagnosis when a young woman presents with fever
and cervical lymphadenopathy.
Digastric Muscle
Intraoperative Image
Radiologic Findings
Initially described in 1972, Kikuchi-Fujimoto disease is a rare and
uncommon clinical condition most commonly affecting young women
in 3rd and 4th decades and presents typically as cervical lymphadenitis
with low-grade fever, malaise, and fatigue although generalized disease
has been reported. The differential diagnosis is broad and disease can
mimic as lymphoma, tuberculosis, metastatic disease, SLE, cat scratch
disease and infectious mononucleosis.
Definitive diagnosis is made by lymph node biopsy showing unique
patchy, irregular areas of eosinophilic necrosis in the paracortex. The
disease is self-limited and symptoms resolve within 1-6 months with no
real effective treatment.
Case Presentation
Our patient is a 30-year-old female of Indian origin with no significant
past medical history who initially presented to her PMD for neck pain,
swelling, fever and fatigue that was treated as upper respiratory infection
with antibiotics. With no improvement in her symptoms and increased
neck swelling with palpable lymph nodes, an ultrasound and CT scan
was done showing extensive right neck lymphadenopathy and an 8 x 6
mm right thyroid nodule. Blood work showed elevated monocytes and
elevated TSH of 13.36 mIU/ml.
Right Neck dissection
Sagittal CT View
of Neck showing
right thyroid
nodule – 6 mm
Coronal CT View
of Neck showing
right sided level V
Transverse CT View
of Neck showing
right thyroid nodule
and right-sided
Neck Ultrasound
showing 6 x 8 mm
right thyroid
Pathologic Findings
Papillary Carcinoma of
thyroid gland
Metastatic carcinoma of the
lymph node
Histiocytic necrotizing
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