Case Report
Open Access
Papillary thyroid carcinoma coexists with undifferentiated
carcinoma & A case report and review of literature
Ren Chongxi*
Cangzhou Clinical College of Integrated Traditional Chinese and Western Medicine of Hebei Medical University, Cangzhou City, Hebei Province, China
*Corresponding author:Ren Chongxi, Cangzhou Clinical College of Integrated Traditional Chinese and Western Medicine of Hebei Medical University,
Qian Tong North Street NO.17 Cangzhou City, Hebei Province, China, Phone Number: +86-18031782233; E-mail:
@
Received: 17 September, 2016; Accepted:03 November, 2016; Published:14 November, 2016
Citation: Chongxi R (2016) Papillary thyroid carcinoma coexists with undifferentiated carcinoma & A case report and review of
literature. SOJ Surgery 3(3): 1-3. DOI: http://dx.doi.org/10.15226/2376-4570/3/3/00134
Abstract
Coexistence of Papillary Thyroid Carcinoma (PTC) with
Undifferentiated Thyroid Carcinoma (UTC) is extremely rare.
The etiopathogenesis has not been elucidated though it may be
associated with de differentiation. we report a case of an 89 yearold
women with a painless mass of the right neck with 5 months.
The patient underwent a lobectomy. The histology showed
that the tumor was composed of epithelioid spindle cells with
hyperchromatic, karyokinesis obviously and abundant blood sinus.
Immunohistochemical studies demonstrated the tumor cells to be
Vimentin (+), P53 (+), CK19 (+), CK516 (+), TTF-1 (-), TG (-), CD34
(-) and Ki-67 (+20 %). The patient still alive after 6 months? It is a
challenging diagnosis to make due to lack of the recognition. Before
making this diagnosis, it is important to strengthen awareness of the
disease, especially for the elderly. And this case may be provided
further evidence and images of this dedifferentiation
Introduction
Papillary thyroid carcinoma (PTC) is the commonest thyroid
carcinoma worldwide [1], while undifferentiated thyroid
carcinoma (UTC) , a term we will use as a synonym for anaplastic
thyroid carcinoma (ATC), ranks among the most lethal of all
human malignancies, and represents nowadays less than 3% of
all clinically recognized malignant thyroid neoplasms [2]. PTC,
as differentiated thyroid carcinoma (DTC), has good prognosis
but 5% of the patients already have distant metastasis at the
diagnosis [3]. The incidence of PTC was about 83.7% in thyroid
malignancies and increased 5.2% per year, according to the
epidemiologic data by Cavalheiro BG et al. [4] And UTC is a rare
and extremely aggressive malignancy, with the median survival
of less than 6 months due to rapid progression and resistance
to multimodal therapies [5]. However, coexistence of PTC with
UTC is extremely rare. To the best of our knowledge, very little
is known about this condition and no authoritative prognostic
data exist. The present study reports the case of an 89 year old
female patient diagnosed as coexistence of PTC with UTC and the
relevant literature is comprehensively reviewed.
Case report
We present a case of an 89 year old female with a rapidly
growing and painless neck mass over a period of five months.
No evidence of dyspnea or dysphagia was identified. Her past
medical history includes a multinodular goiter diagnosed two
years ago with no follow-up. Palpation revealed a large, irregular,
firm, tender and painless tumor mass in the right anterior neck.
She was clinically euthyroid with normal plasma level of thyroid
stimulating hormones. Ultrasonography of the thyroid identified
a 7.1 x 5.3 cm, ill defined hypoechoic mass arising from the right
thyroid lobe. CT revealed an ill defined, low density mass in the
right thyroid lobe that was adjacent to carotid artery. A total
body scan did not show any other mass. The patient underwent
a surgical exploration. There was a hard, intact capsule tumor of
the whole right lobe of the thyroid gland, which was attached to
the right carotid sheach. Subsequently, a right thyroid lobectomy
was performed. The resected lobe measured 5.5 cm in the largest
dimension; No lymph node metastasis was identified.
The resected specimen (4-μm) was fixed in 10% buffered formalin (Sigma Aldrich, St. Louis, MO, USA), processed and embedded in paraffin (Leica, Mannheim, Germany) using standard histological methods. Staining was visualized using an inverted microscope (TE2000-U; Nikon Corporation, Tokyo, Japan). Hematoxylin and eosin (Sigma Aldrich) staining revealed that epithelioid spindle cells with hyperchromatic, karyokinesis obviously and abundant blood sinus were seen in the tumor [Figure 1]. Immunohistochemistry was performed. The monoclonal mouse anti‑human vimentin (clone, V9; cat. no. AX074-YCD; dilution, 1:200; BioGenex, USA) was used for vimentin staining. The monoclonal mouse anti‑human Ki 67 antigen (clone, MIB‑1; cat. no. IR626; dilution, 1:200; Dako) was used for Ki 67 staining. The monoclonal mouse anti human cytokeratin (clone, AE1/AE3; cat. no. IR620; dilution, 1:100; Dako) was used for cytokeratin staining. Immunohistochemical staining was positive for CK19, CK516, p53, vimentin and Ki 67 (20%), and negative for TTF-1, TG and CD34 [Figure 2]. The final diagnosis was papillary thyroid carcinoma coexisting with undifferentiated thyroid carcinoma.
The resected specimen (4-μm) was fixed in 10% buffered formalin (Sigma Aldrich, St. Louis, MO, USA), processed and embedded in paraffin (Leica, Mannheim, Germany) using standard histological methods. Staining was visualized using an inverted microscope (TE2000-U; Nikon Corporation, Tokyo, Japan). Hematoxylin and eosin (Sigma Aldrich) staining revealed that epithelioid spindle cells with hyperchromatic, karyokinesis obviously and abundant blood sinus were seen in the tumor [Figure 1]. Immunohistochemistry was performed. The monoclonal mouse anti‑human vimentin (clone, V9; cat. no. AX074-YCD; dilution, 1:200; BioGenex, USA) was used for vimentin staining. The monoclonal mouse anti‑human Ki 67 antigen (clone, MIB‑1; cat. no. IR626; dilution, 1:200; Dako) was used for Ki 67 staining. The monoclonal mouse anti human cytokeratin (clone, AE1/AE3; cat. no. IR620; dilution, 1:100; Dako) was used for cytokeratin staining. Immunohistochemical staining was positive for CK19, CK516, p53, vimentin and Ki 67 (20%), and negative for TTF-1, TG and CD34 [Figure 2]. The final diagnosis was papillary thyroid carcinoma coexisting with undifferentiated thyroid carcinoma.
Figure 1: Histopathological examination revealing that the tumor was
composed of epithelioid spindle cells with hyperchromatic, karyokinesis
obviously and abundant blood sinus (stain, hematoxylin and eosin;
magnification, x10)
Figure 2: Immunohistochemical staining showed as follows: Vimentin
(+), P53 (+), CK19 (+), CK516 (+), TTF-1 (-), TG (-), CD34 (-), Ki-67 (+ 20
%). The final diagnosis was papillary thyroid carcinoma coexisting with
undifferentiated thyroid carcinoma (magnification, x10)
Discussion
Thyroid cancer is the most common endocrine malignancy
and its incidence goes on increasing worldwide. The majority
of thyroid tumours comprise well-differentiated (papillary and
follicular) thyroid carcinomas that usually carry an excellent
prognosis, while a minority progress to poorly differentiated
carcinoma [6]. PTC is the commonest thyroid cancer, while UTC
is one of the most aggressive and deadly cancers in humans
and accounts for one to two cases per million persons annually.
However, PTC coexisting with UTC of the thyroid is exceedingly
rare. It is noteworthy that UTC may display a very polymorphic
microscopic features, including spindle cells that can mimic
the microscopic appearance of fibrosarcoma, leiomyosarcoma,
or malignant fibrous histiocytoma. Epithelial markers can be
missing in approximately 20% of cases [7] and even more often
in spindle cell sarcomatoid UTCs. Vimentin is also expressed
in more than 50% of UTCs [8]. PTC is typically nonaggressive
tumors with the survival for stage I disease approximating
100% [9]. It shows an indolent clinical course, with localized
disease commonplace, and typically do not recur or metastasize
beyond local lymph nodes. Therefore, a simple thyroidectomy
is of entimes curative for the lesion.UTC accounts for 1%-2% of
all thyroid tumors, with the characteristics of fast progression,
strong local invasion, high distant metastasis rate and so on.
The tumor has a very poor prognosis, and the average survival
time is approximately 6 to 8 months [10]. Multimodal therapy combining surgery, chemotherapy and radiation therapy, might
achieve better results in improving survival in some patients;
however, UTC has a very low cure rate even with the extremely
radical treatments
In our case, the diagnosis of PTC coexisting with UTC was made on the pathological, and immunohistochemical features of the tumors, which were similar to those found in the literature [11]. Despite surgical excision, most patients die after 3 to 6 months. As to UTC, the rarity of this malignancy and the rapidity by which it grows has been a major barrier to progress in finding effective therapies. Thus, the treatment that is the current standard of care for these patients is largely palliative, and few are cured. A recent study by Antonelli A et al. [12] shows that the CLM3 (a pyrazolo[3,4-d]pyrimidine compound), as an agent with antitumor and antiangiogenic activity, is very promising in the treatment of UTC, opening the way to a future clinical evaluation. The present case study has been reported, in which the patient was still alive with no evidence of disease, with a follow-up of 6 months. As for the etiology, we agree with Eloy C et al and Evans WD [6,11]. And this case may be provided further evidence and images of this dedifferentiation.
In our case, the diagnosis of PTC coexisting with UTC was made on the pathological, and immunohistochemical features of the tumors, which were similar to those found in the literature [11]. Despite surgical excision, most patients die after 3 to 6 months. As to UTC, the rarity of this malignancy and the rapidity by which it grows has been a major barrier to progress in finding effective therapies. Thus, the treatment that is the current standard of care for these patients is largely palliative, and few are cured. A recent study by Antonelli A et al. [12] shows that the CLM3 (a pyrazolo[3,4-d]pyrimidine compound), as an agent with antitumor and antiangiogenic activity, is very promising in the treatment of UTC, opening the way to a future clinical evaluation. The present case study has been reported, in which the patient was still alive with no evidence of disease, with a follow-up of 6 months. As for the etiology, we agree with Eloy C et al and Evans WD [6,11]. And this case may be provided further evidence and images of this dedifferentiation.
Conclusion
In summary, PTC coexisting with UTC of the thyroid is
extremely rare. It is a challenging diagnosis to make due to lack
of the recognition. Before making this diagnosis, it is important
to strengthen awareness of the disease, especially for the elderly.
And this case may be provided further evidence and images of
this dedifferentiation.
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