Metastases Of Unknown Primary Origin Research Articles

DOG1 expression in neuroendocrine neoplasms: Potential applications and diagnostic pitfalls

Neuroendocrine neoplasms represent a heterogeneous group of rare tumors, more frequently arising from gastroenteropancreatic tract and lungs. At the time of diagnosis, 20% of cases are metastatic, and 10% of cases are considered as cancer of unknown primary origin. Several immunohistochemical markers are routinely used to confirm the neuroendocrine differentiation, first among all Synaptophysin and Chromogranin-A; on the other hand, different immunohistochemical markers are used to establish primary anatomical site, as TTF1, CDX2, Islet-1 and Calcitonin, but no marker is available in order to distinguish among different sites of the digestive tract.DOG1 (discovered on GIST-1) is a gene normally expressed in interstitial cells of Cajal and, in routine practice, DOG1 immunostaining is used in diagnosis of GIST (gastrointestinal stromal tumor). DOG1 expression has been described in several neoplasms other than GIST, both in mesenchymal and epithelial neoplasms. In the present study, DOG1 immunostaining has been performed in a large cohort of neuroendocrine neoplasms, including neuroendocrine tumors and neuroendocrine carcinomas, in order to evaluate frequency, intensity and pattern of expression in different anatomical site and in different tumor grade. DOG1 expression was detected in a large percentage of neuroendocrine tumors, with statistically significant association between DOG1 expression and gastrointestinal tract neuroendocrine tumors. As a consequence, DOG1 could be included in marker panel for the identification of primary site in neuroendocrine metastases of unknown primary origin; moreover, these results recommend careful evaluation of DOG1 expression in gastrointestinal neoplasms, in particular in differential diagnosis between epithelioid GIST and neuroendocrine tumors.

An unusual case of a tibial metastasis as the first clinical presentation of a serous papillary carcinoma of the ovarian epithelial type of the testis.

Among testicular neoplasms, ovarian-type epithelial tumors constitute an exceedingly rare group, with only a few cases reported in the literature. We describe the case of an 82-year-old man, presented with complaints of right leg pain and difficulty walking, who was found to have a large right tibial metastasis of unknown primary origin. Whole body CT scan did not reveal any cranial, thoracic or abdominal tumor masses, but it showed abnormal para-aortic lymph nodes and right spermatic cord swelling. An extemporary ultrasound examination found a right testicular mass. The patient underwent radical orchiectomy, and the diagnosis of a serous papillary carcinoma of the ovarian epithelial type of the testis was made. To the best of our knowledge, this is the first case in the literature of isolated bone metastasis from ovarian-type epithelial tumor of testis.

Liver Neuroendocrine Metastases of Unknown Primary Origin. Our Experience in 22 Years at Terciary University Hospital

Liver Neuroendocrine Metastases of Unknown Primary Origin. Our Experience in 22 Years at Terciary University Hospital

Effective examination methods for identifying the primary origins of metastatic bone tumors of unknown primary origin during the initial visit: A retrospective chart review study

Objective:To provide appropriate treatment for patients, early diagnosis of the primary origin of skeletal metastases of unknown primary origin is important. This study aimed to assess the examination strategy effective for identifying the primary origin of skeletal metastases of unknown primary origin.Methods:Sixty-one patients with skeletal metastases of unknown primary origin were reviewed. The primary origin was examined via physical examination, blood test including tumor markers, chest radiography, thoracoabdominal computed tomography scan, positron emission tomography–computed tomography scan, metastatic lesion biopsy, and other assessments. Examination methods considered effective for the diagnosis of the primary origin in a specific type of cancer were investigated.Results:The lung was the most common primary origin site, followed by the lymph nodes, prostate, and breast. Meanwhile, biopsy was the most effective examination, followed by positron emission tomography–computed tomography scan and thoracoabdominal computed tomography scan. Blood tests are useful for detecting hematological malignancies and prostate cancer. Computed tomography scans can be used to identify cancers in the lung, breast, and kidney, which are the common primary origins. Forty-one (67.2%) of the 61 patients with skeletal metastases of unknown primary origin were diagnosed via the first four steps, that is, physical examination, blood test, chest radiography, and thoracoabdominal computed tomography scan. Finally, two patients were diagnosed with skeletal metastases of unknown primary origin.Conclusion:The examination steps used in this study, including physical examination, blood test including tumor markers, chest radiography, thoracoabdominal computed tomography scan, positron emission tomography–computed tomography scan, biopsy, and other assessments were effective in determining the primary origin of skeletal metastases of unknown primary origin during the initial visit.

ML-4 A case of primary CNS lymphoma diagnosed 5 years after stereotactic radiosurgery

Central nervous system primary malignant lymphoma (PCNSL) is rarely diagnosed as multiple metastatic brain tumors. Almost tumors recure early after receiving stereotactic radiosurgery (SRS). Regardless of the fact, the following case report displays PCNSL, diagnosed five years after the initial treatment with SRS as brain metastases of unknown primary origin. This extraordinal case suggests long-term follow-up regarding PCNCL. The case was a 55-year-old woman with a history of a total hysterectomy for cervical cancer. She developed left paralysis. Brain MRI confirmed a 27 mm contrast-enhanced lesion in the right frontal lobe and three other lesions. SRS was performed as a diagnosis of multiple brain metastases for urgent symptom relief. No extra-cranial cancerous lesions were found. Unknown primary cancer was a probable diagnosis at that time. Two years after SRS, local regrowth of tumor of the right frontal primary motor area was discovered. Re-irradiation was performed. Cerebral edema, contrast enhancement, and left paralysis progressed following five months, taking an oral corticosteroid. Craniotomy and debulk. The pathological diagnosis was brain radiation necrosis due to no viable tumor cells. New lesions in the left temporal lobe and basal ganglia appeared three years after surgery. Awake craniotomy was performed for the left temporal lobe lesion. Histopathology showed diffuse growth of tumor cells with a high nucleo-cytoplasmic ratio and irregular nuclear shape. Immunohistochemistry revealed positive CD10, CD20, CD45 (LCA), MUM1, and negative CD3, CD5. The Ki- 67 labeling rate was as high as almost 100% to diagnose diffuse large B-cell lymphoma, PCNSL. Multidrug chemotherapy consisting of rituximab, high-dose methotrexate, procarbazine, and vincristine were performed. Complete remission was obtained without any serious adverse events. Considering the residual radiation necrosis, whole-brain irradiation was avoided. Moreover, consolidation therapy was performed only with high-dose cytarabine therapy.

The impact of 18F-FDG PET/CT in the clinical management of patients with lymph node metastasis of unknown primary origin.

The aim of this study was to evaluate the diagnostic performance and the utility of F-18-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET/CT) in the clinical management of patients presenting with lymph node metastasis of undefined primary origin (CUP). A total of 53 patients (34 males, 19 females) with a diagnosis of lymph node metastasis according to the histopathology and/or conventional imaging were enrolled in this retrospective study. Patients were divided into four groups according to the initial location of their metastasis - group 1, cervical lymph nodes (n=39), group 2, axillary lymph nodes (n=6), group 3, mediastinal lymph nodes (n=2) and group 4, abdominal and pelvic lymph nodes (n=6). The site of a probable primary malignancy suggested by PET/CT was confirmed by biopsy/further investigations or follow-up. 18F-FDG PET/CT accurately detected the primary carcinoma in 19 of 53 patients (36%), with head and neck cancer and lung carcinoma being the most common primary locations. The PET-CT scan results were negative for primary site localization in 13% of patients (false-negative), while 45% had true negative results, and 6% displayed false-positive results. Additional distant metastatic foci were identified in 21 of all patients (40%). The overall sensitivity, specificity, and accuracy rates of the study were identified as 73%, 89%, and 81%, respectively; in the group with cervical lymph node metastasis sensitivity 70% and specificity 84%. To conclude, 18F-FDG PET/CT is a sensitive and selective procedure for detecting unknown primary tumors, especially in the clinical setting of cervical lymph node metastasis and its use should be encouraged earlier in the pre-treatment phase of CUP-patients, leading to higher detection of probable primary sites, guiding subsequent biopsy, and more accurate detection of distant metastases in a single examination.

Failure patterns of cervical lymph nodes in metastases of unknown origin according to target volume.

PurposeThis study was aim to evaluate the patterns of failure according to radiotherapy (RT) target volume for cervical lymph nodes in metastases of unknown primary origin in head and neck region (HNMUO).Materials and MethodsSixty-two patients with HNMUO between 1998 and 2016 were retrospectively reviewed. We analyzed the clinical outcomes and primary site failure depending on the radiation target volume. The target volume was classified according to whether the potential head and neck mucosal sites were included and whether the neck node was treated involved side only or bilaterally.ResultsPotential mucosal site RT (mucosal RT) was done to 23 patients and 39 patients did not receive mucosal RT. Mucosal RT showed no significant effect on overall survival (OS) and locoregional recurrence (LRR). The location of primary site failure encountered during follow-up period was found to be unpredictable and 75% of patients with recurrence received successful salvage therapies. No significant differences in OS and LRR were found between patients treated to unilateral (n = 35) and bilateral neck irradiation (n = 21). Treatment of both necks resulted in significantly higher mucositis. conclusionsWe found no advantages in OS and LRR of patients with HNMUO when mucosal sites and bilateral neck node were included in the radiation target volume.

Tumor control of cervical lymph node metastases of unknown primary origin: the impact of the radiotherapy target volume.

Debate on the extent of treatment of neck metastasis of cancer of unknown primary tumors (CUPs) is still ongoing. In two Dutch tertiary referral centers, the post-surgical radiation target volume changed from the bilateral neck including the pharyngeal axis to the unilateral neck only, in the course of the last decade. This study aims to investigate the outcome of patients with CUP before and after de-escalation of post-surgical radiotherapy. Data of two Dutch tertiary referral centers were merged. Disease-free survival (DFS), overall survival (OS), and regional control rate (RCR) of 80 patients diagnosed with CUP (squamous cell and undifferentiated carcinomas) between 1990 and 2009 were retrospectively analyzed. Thirty patients received bilateral neck and pharyngeal axis radiotherapy and 42 patients ipsilateral radiotherapy only. In another eight patients, the postsurgical radiation target volume was expanded to the contralateral neck or to the pharyngeal axis, due to suspicious lesions on imaging. The 5-year DFS, OS and RCR were 60%, 51.2%, and 80%, respectively, in the total patient population. RCR did not differ in patients treated with ipsilateral as compared to bilateral radiotherapy nor did 5-year OS and DFS. No tumors occurred in the pharyngeal axis. In this study, omittingelective treatment of the contralateral neck and pharyngeal axis did not lead to a decrease in locoregional control or survival rates when treating patients with CUP.

EP1.01-106 A Special Case of Synchronous Multiple Primary Lung Cancer with Mediastinal Lymph Node Metastases of Unknown Primary Origin

EP1.01-106 A Special Case of Synchronous Multiple Primary Lung Cancer with Mediastinal Lymph Node Metastases of Unknown Primary Origin

Role of 18F-FDG PET/CT in the detection of primary malignancy in patients with bone metastasis of unknown origin

ObjectiveThe present study investigates the role of 18F-FDG PET/CT in the detection of primary malignancy in patients with bone metastasis of unknown primary origin (BMUO). Material and MethodsThis retrospective study considered histopathological examination findings or clinical follow-up data as the standard reference in the diagnosis of primary tumors, and results were compared with results of PET/CT scans. ResultsThe study included 100 patients with BMUO (74 males, 26 females, mean age 61 years). The primary origin was identified in 92 of the 100 patients. Adenocarcinoma was the most common histopathological subtype in patients in whom the primary origin of tumor was detected (65.2%). The most common primary tumor was lung carcinoma (n = 52) followed by prostate (n = 13), breast (n = 7), colon (n = 4), gastric (n = 3), ovarian (n = 2), renal cell (n = 2), adrenal(n = 1), thyroid (n = 1), endometrial (n = 1) and parotid (n = 1) carcinoma, hepatobiliary cancers (n = 2), leiomyosarcoma (n = 2) and maxillary sinus tumor (n = 1). The numbers of patients in whom PET/CT showed true positive, true negative, false positive (FP) and false negative (FN) results were 72, 7, 8 and 13, respectively. The sensitivity, specificity, accuracy and detection rate of PET/CT in detecting the primary tumor were 84.7%, 46%, 79% and 72%, respectively. The overall survival was significantly lower in lung cancer group when compared to non-lung cancer group, whereas it was significantly higher in prostate cancer group than in non-prostate cancer group. ConclusionsPET/CT, as a non-invasive method, can be preferred as the first choice in the detection of primary tumor in patients with BMUO.

Patterns of EBV-positive cervical lymph node involvement in head and neck cancer and implications for the management of nasopharyngeal carcinoma T0 classification

ObjectivesEpstein-Barr virus (EBV)-positive cervical lymph node (CLN) metastasis of unknown primary origin is classified as nasopharyngeal carcinoma (NPC) T0 by the American Joint Committee on Cancer staging manual (8th edition). We aimed to investigate the possible primary sites and patterns of EBV-positive CLN metastases and to provide implications for the management of NPC T0 classification. Materials and methodsWe retrospectively reviewed 269 patients with newly diagnosed EBV-positive CLN metastatic disease who underwent EBV detection via EBV-encoded RNA in situ hybridization. Fifteen patients with unknown primary tumors underwent follow-up after initial treatment. ResultsIn patients with EBV-positive CLNs, the most common primary sites after the nasopharynx (51.7%) were the salivary gland (24.5%), lung (7.8%), oropharynx (3.3%), nasal cavity/maxillary (3.3%), oral cavity (2.2%), orbit (1.1%), and liver (0.4%). No primary site was found in 15 patients (5.6%). For salivary gland malignancies, level II and I were the most frequently involved regions. Tumors arising from the lung or liver metastasized to the lower neck (level IV, V, and VI) rather than the upper neck. After initial treatment, 2/15 patients with EBV-positive CLNs of unknown primary exhibited primary NPC and oropharyngeal tumor, respectively. Further, even without prophylactic irradiation to the nasopharynx, only one of 13 unknown primary patients developed NPC. ConclusionsThe origins of EBV-positive CLNs may not be restricted to the nasopharynx alone, and are likely to involve the head and neck or non-head and neck regions. NPC T0 classification should be cautiously assigned to such tumors.

Clinical Utility of In Situ Hybridization Assays in Head and Neck Neoplasms.

Head and neck pathology present a unique set of challenges including the morphological diversity of the neoplasms and presentation of metastases of unknown primary origin. The detection of human papillomavirus and Epstein-Barr virus associated with squamous cell carcinoma and newer entities like HPV-related carcinoma with adenoid cystic like features have critical prognostic and management implications. In salivary gland neoplasms, differential diagnoses can be broad and include non-neoplastic conditions as well as benign and malignant neoplasms. The detection of specific gene rearrangements can be immensely helpful in reaching the diagnosis in pleomorphic adenoma, mucoepidermoid carcinoma, secretory carcinoma, hyalinizing clear cell carcinoma and adenoid cystic carcinoma. Furthermore, molecular techniques are essential in diagnosis of small round blue cell neoplasms and spindle cell neoplasms including Ewing sarcoma, rhabdomyosarcoma, synovial sarcoma, biphenotypic sinonasal sarcoma, dermatofibrosarcoma protuberans, nodular fasciitis and inflammatory myofibroblastic tumor. The detection of genetic rearrangements is also important in lymphomas particularly in identifying 'double-hit' and 'triple-hit' lymphomas in diffuse large B cell lymphoma. This article reviews the use of in situ hybridization in the diagnosis of these neoplasms.

GATA3 as a putative marker of breast cancer metastasis-A retrospective immunohistochemical study

Diagnostic verification of breast cancer metastasis with histopathology and imaging analysis is essential to determine tumor staging. The aim of this study was to validate the utility of GATA3 immunohistochemistry as a diagnostic marker for breast cancer metastases and metastases of unknown primary origin. Retrospective immunohistochemical analysis of GATA3 expression in 164 breast cancer metastases diagnosed between 2004 and 2014 showed a striking difference between mammaglobin and GATA3 expression (51.2% vs 94% positivity). These findings highlight GATA3 as a more reliable and sensitive diagnostic marker for breast cancer metastases and possibly metastatic tumors of unknown origin than mammaglobin.

Optimal Treatment Decision for Brain Metastases of Unknown Primary Origin: The Role and Timing of Radiosurgery

BackgroundUp to 15% of all patients with brain metastases have no clearly detected primary site despite intensive evaluation, and this incidence has decreased with the use of improved imaging technology. Radiosurgery has been evaluated as one of the treatment modality for patients with limited brain metastases. In this study, we evaluated the effectiveness of radiosurgery for brain metastases from unknown primary tumors.MethodsWe retrospectively evaluated 540 patients who underwent gamma knife radiosurgery (GKRS) for brain metastases radiologically diagnosed between August 1992 and September 2007 in our institution. First, the brain metastases were grouped into metachronous, synchronous, and precocious presentations according to the timing of diagnosis of the brain metastases. Then, synchronous and precocious brain metastases were further grouped into 1) unknown primary; 2) delayed known primary; and 3) synchronous metastases according to the timing of diagnosis of the primary origin. We analyzed the survival time and time to new brain metastasis in each group.ResultsOf the 540 patients, 29 (5.4%) presented precocious or synchronous metastases (34 GKRS procedures for 174 lesions). The primary tumor was not found even after intensive and repeated systemic evaluation in 10 patients (unknown primary, 34.5%); found after 8 months in 3 patients (delayed known primary, 1.2%); and diagnosed at the same time as the brain metastases in 16 patients (synchronous metastasis, 55.2%). No statistically significant differences in survival time and time to new brain metastasis were found among the three groups.ConclusionIdentification of a primary tumor before GKRS did not affect the patient outcomes. If other possible differential diagnoses were completely excluded, early GKRS can be an effective treatment option for brain metastases from unknown primary tumor.

Management of neck metastases of unknown primary origin united in two European centers

Combined analysis of diagnostic and therapeutic management of neck metastases of carcinoma of unknown primary origin ('true CUP') in two European tertiary referral centers (University Medical Centers of Maastricht, NL and Cologne, D) to contribute to the ongoing discussion on management in CUP. Retrospective analysis of 29 (Maastricht) and 22 (Cologne) true cervical CUP syndrome patients (squamous cell carcinoma). The diagnostic and therapeutic approaches were correlated with clinical follow-up data and HPV status. In total, 48 out of 51 true CUP patients received postsurgical adjuvant radiotherapy. In eight patients from Cologne, this was combined with concomitant platin-based chemotherapy. Neither in Cologne nor in Maastricht, radiotherapy of the pharyngeal mucosa was commonly performed (n = 6, 12.5 %) The percentage of patients who were irradiated ipsilaterally or bilaterally did not differ between both institutes (N = 21/27 in Maastricht vs. 11/21 in Cologne), nor did the 5-year overall survival differ significantly. Oncogenic HPV was only found in 4 out of 51 CUPs (7, 8 %). Therefore, no relation with overall and recurrence-free survival could be detected. No occult primary tumors were revealed during follow-up despite de-escalation of therapy by abandoning irradiation of the pharyngeal mucosa in both institutes. There were no significant differences between ipsilateral and bilaterally irradiated patients regarding overall and recurrence-free survival. The occurrence of distant metastases was more often noticed in ipsilaterally treated patients as compared to bilaterally radiated patients (8 vs. 2, p = 0.099). Those patients all had been classified N2b or higher. International guidelines still are not unified and there is an urgent need for a consented therapeutic regimen. Comparison of two international strategies on the management of CUP patients is presented and further research is recommended regarding the role of radiotherapy of the pharyngeal axis, the value of unilateral and bilateral radiotherapy and the role of concomitant or induction chemotherapy in CUP patients, particularly in N2b or higher-staged neck disease. The prevalence and role of HPV in true CUP after thorough diagnostic work-up seem limited in our case series, particularly when compared to the role in oropharyngeal carcinomas.

The Value of Molecular Analysis for the Workup of Metastasis of Unknown Primary Origin

AbstractMetastasis of unknown primary origin occurs in approximately 2% to 10% of all noncutaneous malignancies and makes up approximately 10% of new referrals to the hospital-based oncology centers. Determination of the primary site is based on histologic and cytologic examination, ancillary studie

Diagnostic Value of 68Ga-DOTATATE PET/CT in Liver Metastases of Neuroendocrine Tumours of Unknown Origin

In neuroendocrine liver metastases of unknown primary, a multimodality approach is usually adopted and consists of transabdominal ultrasound, endoscopic ultrasound (EUS), computed tomography (CT), magnetic resonance imaging (MRI), nuclear medicine techniques, endoscopy and exploratory surgery. The purpose of the study is to evaluate the diagnostic value of (68)Ga-DOTATATE positron emission tomography (PET)/CT as part of a multimodality approach in neuroendocrine liver metastases of unknown primary. Six patients (M:F = 5:1, age range 28-56 years) with immunohistochemically proven neuroendocrine liver metastases but inconclusive initial CT work-up were retrospectively analysed. Clinical finding, histopathology, comparative imaging and follow-up were used to validate the results when ethically justified. (68)Ga-DOTATATE PET/CT identified the primary tumour in five out of six (83.3%) patients: pancreas (n = 4) and stomach (n = 1). Out of three patients with indeterminate primary on initial CT, two patients were confirmed by (68)Ga-DOTATATE PET/CT. Absence of uptake in indeterminate primary of one patient was later confirmed negative by histopathology. In another three patients with undetected primary on initial CT, primary site was demonstrated in all patients with unsuspected metastases in two patients on (68)Ga-DOTATATE PET/ CT. No further work-up was done to confirm the primary in patients with distant metastases. Change of management was observed in three out of six (50%) patients. Our small study indicates that (68)Ga-DOTATATE PET/CT is a promising diagnostic option in the multimodality approach to neuroendocrine liver metastases of unknown primary origin.

OP128: Differences in management of neck metastases of unknown primary origin in two european centres: Consequences for future strategies

Purpose Analysis of diagnostic and therapeutic management of neck metastasis of unknown primary origin (“true”-CUP) in two European tertiary referral centers (University Medical Centers of Maastricht and Cologne), and the presence of human papillomavirus (HPV) in this population. Materials and methods Retrospective analysis of 29 (Maastricht) and 22 (Cologne) “true”-CUP-patients (squamous cell carcinoma) for which an intended curative neck dissection was performed. The diagnostic and therapeutic approach were correlated with clinical follow-up data and HPV-status. Results In Maastricht, PET-CT (whole body) was performed since 2002, whereas in Cologne FDG-PET without CT was routinely performed since 2006 next to CT and ultrasound of the abdomen and skeletal scintigraphy, however with no additional information regarding tumor spreading. Oncogenic HPV was found exclusively in 18% of CUPs from Cologne (18 vs. 0%, p = 0.017). However, no differences were noted for recurrent disease or survival compared to HPV-negative CUPs. In Maastricht, unilateral adjuvant radiotherapy of the neck without involvement of the pharyngeal axis was performed more often ( n = 22/29) compared to bilateral radiotherapy including the pharyngeal axis ( n = 20/22) and combined with chemotherapy in Cologne ( n = 8/22). No primary tumors arose during five years of follow-up in Maastricht or Cologne. CUPs from Maastricht more frequently developed contralateral regional recurrences (5 vs. 0 patients: log rank p p = 0.099). The five-year-overall survival did not differ significantly. Conclusions The more extensive diagnostic approach (ultrasound/CT abdomen and skeletal scintigraphy) was not of additional value, neither did HPV-status contribute on primary tumor location or survival. De-escalation of therapy by abandoning irradiation of the pharyngeal axis did not lead to diminished local control. Contralateral irradiation offers potential advantages for regional control, particularly in the pN2b- and pN3-group.

Bilateral Synchronous Tonsillar Carcinoma

Bilateral Synchronous Tonsillar Carcinoma Introduction: The tonsils are the most common site of occult primaries in the oropharynx with a reported incidence of 18%-44%. Bilateral synchronous tonsillar carcinoma has been reported 15 times in the literature however the incidence is likely to be much higher as bilateral tonsillectomy in the work-up of a patient with cervical metastases of unknown primary origin is not routine practice in all centres. Methods: A case series of four patients presenting with metastatic squamous cell carcinoma (SCC) in cervical lymph nodes with unknown primaries found on bilateral tonsillectomy to have bilateral synchronous tonsillar carcinoma. Discussion: This cases series supports the case for bilateral tonsillectomy to become standard practice in the work-up of a patient with cervical metastases of unknown primary origin.

Angiosarcoma of the scalp diagnosed by the presence of neck inflammation: a case report

Angiosarcoma is an uncommon malignancy, which spread out from the endothelial cells of vessels. Scalp angiosarcoma with cervical lymph node metastasis is particularly rare. This article describes a rare case of angiosarcoma of the scalp, presenting as neck inflammation. Imaging procedures such as computed tomography (CT), magnetic resonance image (MRI) and ultrasonography (US) were not sufficient to diagnose this case. A needle biopsy provided an effective and accurate diagnosis of cervical lymph node metastasis. Additional observation and physical examination was required to diagnose the origin of the primary cancerous lesion. Once the angiosarcoma diagnosis was confirmed histologically, sequential weekly and monthly docetaxel (DTX) treatment was effective in preventing reoccurrence. Nonetheless, the optimization of angiosarcoma treatment remains a future goal. Although patients generally describe pain and swelling at the primary lesion site, this patient complained only of painful neck inflammation, without any indication of pain or swelling of the scalp. A revised diagnostic protocol should note that cervical lymph node metastasis of unknown primary origin may result from angiosarcoma of the scalp.