Incidence of cancers of unknown primary origin in India and their trends during 1986–2014: A joinpoint regression analysis

Background: Cancer of unknown primary (CUP) origin is cancer in which malignant cancer cells are in the body but the site of cancer where it began is unknown. Detailed incidence and time trends of these cancers, specific to various regions in India is needed. This paper aims to summarize and report the incidence of other and unknown (O&U) cancers across India in 27 population-based cancer registries (PBCRs) and to study the trends among these cancers using joinpoint regression analysis. Methods: Data on the incidence of CUP were obtained from the published reports on 27 PBCRs of the National Cancer Registry Programme (NCRP) of the Indian Council of Medical Research (ICMR). A joinpoint regression model was used to analyze the long-term trends of incidence related to CUP based on published data from PBCRs between 1986 and 2014. Annual Percent Change (APC) in incidence rate was estimated for various registries. Results: The northeast region had the highest age-adjusted rate (AAR) for both men (1.76–29.7) and women (1.99–14.68). Age-specific rate (ASR) for both men (39.8–855.7) and women (48.2–470.4) was highest in the northeast region. There is an increase in the incidence rate for all six major registries over the past decade with an exception of women in the Delhi Cancer Registry. There is a decline in incidence rate by 0.14 during 1990–2012 in the female population of the Delhi registry. Conclusion: The increasing incidence trends of CUP is a matter of concern for the healthcare professionals and researchers. There is a need for research and advanced and improved diagnostic tools for the improvement of the status of O&U cancers.

Unknown primary tumor sites in spinal metastasis / Metástase vertebral em tumor primário de localização desconhecida / Metástasis vertebral en tumor primario de localización desconocida

ABSTRACT Objective: The goal of the study was to report the prevalence of spinal metastasis with unknown primary tumor, clinical features, treatment results and patient survival. Methods: A retrospective evaluation of 103 patients with spinal metastasis and epidural compression, who underwent surgical treatment between March 2009 and August 2015. The prevalence of metastatic spinal tumor with unknown primary tumor was evaluated, as well clinical features, survival and treatment results. Results: Of 103 patients with spinal metastasis and epidural compression, seven patients (6.8%) with unknown primary tumor site were identified; five (71.4%) male and two (28.6%) female, with ages ranging from 37 to 67 years (50.7 years). The metastasis was located in the thoracic spine in six of the patients (85.7%) and in the cervical spine in one (14.3%). The mean survival time was 44.8 days. Conclusion: Spinal metastasis with unknown primary tumor was found in 6.8% of patients. The prognosis and outcomes were poor, and patients had poor survival rates. Level of evidence III; Experimental study.

RESUMO Objetivo: O objetivo deste estudo foi relatar a prevalência de metástases vertebrais em tumores primários desconhecidos, suas características clínicas, resultados do tratamento e sobrevida dos pacientes. Métodos: Avaliação retrospectiva de 103 pacientes com metástase da coluna vertebral e compressão epidural, submetidos a tratamento cirúrgico entre março de 2009 e agosto de 2015. Avaliou-se a prevalência de tumores metastáticos vertebrais com tumor primário desconhecido, assim como as características clínicas, a sobrevida e os resultados do tratamento. Resultados: Dos 103 pacientes com metástase vertebral e compressão epidural, foram identificados sete pacientes (6,8%) com tumor primário de origem desconhecida; cinco pacientes (71,4%) eram do sexo masculino e dois pacientes (28,6%) do sexo feminino, com idades variando de 37 a 67 anos (50,7 anos). A localização da metástase vertebral era na coluna torácica em seis pacientes (85,7%) e na coluna cervical em um paciente (14,3%). A média de sobrevida dos pacientes foi de 44,8 dias. Conclusões: As metástases vertebrais com tumor primário de origem desconhecida foram observadas em 6,8% dos pacientes. O prognóstico e os resultados foram ruins, e os pacientes tiveram sobrevida bastante baixa. Nível de evidencia III; Estudo Comparativo Retrospectivo.

RESUMEN Objetivo: El objetivo de este estudio fue relatar la prevalencia de metástasis vertebrales en tumores primarios desconocidos, sus características clínicas, resultados del tratamiento y sobrevida de los pacientes. Métodos: Evaluación retrospectiva de 103 pacientes con metástasis de la columna vertebral y compresión epidural, sometidos a tratamiento quirúrgico entre marzo de 2009 y agosto de 2015. Se evaluó la prevalencia de tumores metastásicos con tumor primario desconocido, así como las características clínicas, la sobrevida y los resultados del tratamiento. Resultados: De los 103 pacientes con metástasis vertebral y compresión epidural, fueron identificados siete pacientes (6,8%) con tumor primario de origen desconocido; cinco pacientes (71,4 %%) eran del sexo masculino y dos (28,6%) del sexo femenino, con edades variando de 37 a 67 años (50,7 años). La localización de la metástasis vertebral era en la columna torácica en seis pacientes (85,7%) y en la columna cervical en un paciente (14,3%). El promedio de sobrevida de los pacientes fue de 44,8 días. Conclusiones: Las metástasis vertebrales con tumor primario de origen desconocido fueron observadas en 6,8% de los pacientes. El pronóstico y los resultados fueron malos y los pacientes tuvieron sobrevida bastante baja. Nivel de evidencia III; Estudio Comparativo Retrospectivo.

Melanoma maligno gastrointestinal de origen primario desconocido: reporte de caso / A case report of malignant gastrointestinal melanoma of unknown primary origin

Resumen Una de las características inusuales del melanoma cutáneo es la capacidad de metastatizar en el intestino delgado. A menudo se diagnostica durante la autopsia, en la cual se ha encontrado depósitos metastásicos en el 50%-60% de los casos, pero menos del 2% al 4% de los pacientes con diagnóstico de melanoma tiene metástasis gastrointestinal (GI) durante el curso de la enfermedad y entre el 4%-9% de casos de melanoma GI tiene un primario desconocido. La rápida identificación y resección del melanoma en el tracto digestivo podría mejorar la tasa de supervivencia en aquellos pacientes en los que se presentan complicaciones, como obstrucción intestinal. Se presenta un caso clínico de melanoma gastrointestinal de origen primario desconocido. El porcentaje de este tipo de casos es poco frecuente. La paciente presentó cuadro clínico de náuseas, hiporexia, epigastralgia, fatiga, parestesias en región dorsal derecha y disminución de 9 kilogramos en 3 semanas. Le realizaron tomografía computarizada (TAC) de abdomen contrastado, que reportó 3 lesiones en hígado de predominio isodenso en el parénquima, con algunas áreas de menor densidad al interior, localizadas en los segmentos 2, 5, 7 y 8 del hígado al que se le realizó la biopsia. Se llevó a cabo endoscopia de vías digestivas altas con biopsia de 2 lesiones hiperpigmentadas en la segunda porción de duodeno. El examen histopatológico de las biopsias evidenció melanoma maligno. Se excluyeron todas las localizaciones típicas de melanoma primario durante el procedimiento diagnóstico.

Abstract One of the unusual characteristics of cutaneous melanoma is its ability to metastasize in the small intestine. It is often diagnosed during autopsies of cutaneous melanoma patients. Metastatic deposits have been found in 50% to 60% of these autopsies, but less than 2% to 4% of patients diagnosed with melanoma have gastrointestinal metastasis during the course of the disease. Between 4% and 9% of gastrointestinal melanoma cases have unknown primary tumors. Rapid identification and resection of melanoma in the digestive tract could improve the patient survival rate and prevent complications such as intestinal obstructions from occurring. We present a rare clinical case of gastrointestinal melanoma of unknown primary origin. The patient had a clinical picture of nausea, hyporexia, epigastralgia, fatigue, paresthesias in the right dorsal region and had lost nine kilograms in three weeks. An abdominal CT scan showed three predominantly isodense liver lesions in the parenchyma, with some areas of lower density located in segments 2,5,7 and 8 of the liver. These were biopsied. Upper digestive tract endoscopy took biopsy samples of two hyperpigmented lesions in the second portion of the duodenum. Histopathological examination showed malignant melanoma. All typical locations of primary melanoma were excluded during the diagnostic procedure.

Sarcoma de células claras (melanoma maligno de partes moles): relato de caso / Clear cell sarcoma (malignant melanoma of soft parts): case report

O sarcoma de células claras (SCC) é um tumor maligno descrito em 1965. Essa neoplasia representa menos de 1% dos sarcomas, sendo que esses já representam 1% dos cânceres. O SCC geralmente acomete adultos jovens e apresenta-se como uma massa profunda, localizada em extremidades, próxima a tendões e aponeuroses. O prognóstico é geralmente reservado, com sobrevida em 5 anos de 47%. Nesse artigo é relatado o caso de uma paciente de 38 anos com uma neoplasia metastática sem sítio primário conhecido, que se apresentou inicialmente como uma tumoração em região inguinal. A microscopia da lesão revelou neoplasia composta por células epitelioides de grande porte, citoplasma eosinófilo, comumente contendo pigmento, e núcleo pleomórfico. A imuno-histoquímica (IHQ) foi negativa para pancitoqueratina e desmina e positiva para melanossoma, melan-A, vimentina, S-100 e positiva em 40% das células para Ki-67. O tratamento paliativo foi realizado com quimioterapia e radioterapia. A paciente evoluiu à óbito seis meses após o diagnóstico. O SCC é uma neoplasia maligna rara de difícil diagnóstico diferencial. O exame histológico e mesmo a IHQ podem não conseguir diferenciá-lo do melanoma metastático. A pesquisa da translocação t(12;22)(q13;q13) pode ajudar a diferenciá-los, porém não é feita rotineiramente. Assim, o diagnóstico foi firmado pelas características clínicas, em correlação com a patologia. Devido a sua raridade não há um tratamento clínico bem estabelecido. Considerando que a terapia para o melanoma metastático está em evolução e é cada vez mais efetiva, é importante que o diagnóstico diferencial preciso com o SCC seja feito.

Clear cell sarcoma (CCS) is a malignant tumor described in 1965. This neoplasm accounts for less than 1% of sarcomas, which already account for 1% of cancers. CCS usually affects young adults and presents as a deep mass, located at extremities, near tendons and aponeuroses. The prognosis is generally limited, with a 5-year survival of 47%. In this article, it is reported a case of a 38-year-old patient with metastatic neoplasia of unknown primary site, which initially presented as an inguinal tumor. Microscopy revealed neoplasia composed of large epithelioid cells, eosinophilic cytoplasm, commonly containing pigment, and pleomorphic nucleus. Immunohistochemistry (IHC) was negative for pan-cytokeratin and desmin and positive for melanosome, melan-A, vimentin, S-100 and positive in 40% of cells for Ki-67. Palliative treatment was performed with chemotherapy and radiotherapy. The patient died six months after the diagnosis. CCS is a rare malignant neoplasm of difficult differential diagnosis. Histological examination and even IHC may not be able to differentiate it from metastatic melanoma. The translocation t (12; 22) (q13; q13) may help to differentiate them but it is not routinely researched for. Therefore, the diagnosis was established by correlation of clinical and pathological characteristics. Due to its rarity, there is no well-established clinical treatment. Considering that therapy for metastatic melanoma is evolving and is increasingly effective, it is important that a precise differential diagnosis with SCC is done.

The Results of Occult Breast Carcinoma Treated with Axillary Node Dissection Only: Five Cases Reports

Herein, we report five cases of occult breast cancer treated with axillary node dissection only, without breast surgery or whole breast radio-therapy. The patients complained of a large, hard mass in the axillary area, although no breast masses were palpable. Biopsy of the axillary mass was performed in each case, and histological examination showed a metastatic carcinoma. No malignant findings were observed by mammography or ultrasonography. Magnetic resonance imaging and systemic examinations revealed no extramammary primary lesions. All patients underwent axillary lymph node dissection without breast surgery, and were administered adjuvant chemotherapy but not whole breast radiation therapy. The median follow-up period was 56 months (range, 15–241 months). The patients were all alive with no evidence of disease at the end of the follow-up period.

Undiagnosed Anaplastic Ganglioglioma Resulting in the Sudden Unexpected Death of a Young Woman / 대한법의학회지

Intracranial neoplasms usually have definitive symptoms, causing a need for medical intervention, but a few cases result in sudden unexpected death even before diagnosis. In these cases, autopsy or post-mortem imaging may be the only way of identifying the existence of a tumor, so investigators or forensic specialists who participate in the post-mortem inspection should be aware of these possibilities. We report on a case of a 26-year-old woman without any medical history found dead in her home. A 5-cm intraventricular tumor was found during autopsy, which was histologically consistent with anaplastic ganglioglioma a very rare type of neuroglial tumor with the potential for malignant behavior. The tumor showed the characteristic features of anaplastic ganglioglioma, such as increased cellularity, a high Ki-67 proliferative index, and necrosis. There were signs of increased intracranial pressure, including flattened gyri and dilated ventricles, which led to the conclusion that this brain tumor was the cause of death.

Complete biologic response to taxane based chemotherapy confirmed by 18FFDG PET/CT and surgery in a cancer of unknown primary site / 부인종양

Cancers of an unknown primary site are heterogenous with respect to their clinical and pathologic features. They are generally very aggressive, but specific favorable subsets have a better prognosis. For these favorable subsets, taxane based chemotherapy is very effective for a subset of woman with papillary serous peritoneal adenocarcinoma. A 52 year-old woman underwent [18F]-FDG PET/CT for routine health screening. On PET/CT, multiple hypermetabolic lymph nodes were detected in the paraaortic spaces, and there were no other hypermetabolic abnormalities. The patient was diagnosed with an unknown primary cancer that probably originated from the ovary or peritoneum, according to clinical studies and biopsy results. This was not a typical case of a favorable subset of cancer of an unknown primary site, but the tumor showed complete biologic response to taxane based chemotherapy as revealed by PET/CT, and necrotic tumor cells were confirmed by surgery.

Poorly Differentiated Neuroendocrine Carcinoma in a Perigastric Lymph Node from an Unknown Primary Site / Journal of the Korean Cancer Association, 대한암학회지

Neuroendocrine carcinomas from an unknown primary site are uncommon. The authors report on a case of neuroendocrine carcinoma in a perigastric lymph node (LN) with no primary site. A 52-year-old male patient with early gastric adenocarcinoma underwent treatment by endoscopic submucosal dissection, and, six months later, findings on a computed tomographic scan of the abdomen revealed a LN enlargement measuring 2.0 cm in the perigastric region. The patient underwent subtotal gastrectomy and regional LN dissection under a suggestive preoperative diagnosis of gastric adenocarcinoma with LN metastasis. However, microscopically, no residual tumor was found in the stomach, and the perigastric LN showed poorly differentiated neuroendocrine carcinoma (PDNEC). After an extensive workup, no primary site was identified. The patient also received four cycles of etoposide and cisplatin. Despite its extremely rare incidence, this case suggests that PDNEC of an unknown primary site is limited to a single site, and that resection should be considered in combination with chemotherapy.

Cisto branquial malignizado ou metástase de tumor primário? relato de caso / Malignant branchiogenic cyst or primary tumor metastasis? case report

Introdução: A existência de cisto branquial malignizado é controversa desde a época em que foi descrito pela primeira vez. A maioria dos autores acredita que, na verdade, é uma metástase cística de tumor primário de cabeça e pescoço. Objetivo: Discutir sobre a existência de cisto branquial malignizado. Relato do Caso: Paciente apresentando massa cística anterior à porção superior do músculo esternocleidomastóideo, que foi removida como um cisto branquial malignizado. Entretanto, o exame anátomo patológico sugeriu tratar-se de um tumor primário desconhecido. A paciente vem sendo seguida com exames periódicos sem evidências do primário. Considerações Finais: Provavelmente nunca saberemos se era um cisto branquial malignizado ou metástase de primário desconhecido devido a sobreposição de critérios diagnósticos.

Introduction: The existence of malignant branchiogenic cyst has been controversial since it was first described. Most authors believe that it is actually a head and neck primary tumor cystic metastasis. Objective: Discuss the existence of malignant branchiogenic cyst. Case Report: A patient with a cystic mass in front of the sternocleidomastoideus muscle superior part, which was removed as a malignant branchiogenic cyst. However, the anatomopathological examination suggested it was an unknown primary tumor. The patient has been followed-up with periodic examinations without evidences of a primary tumor. Conclusion: Probably we will never know whether it was a malignant branchiogenic cyst or an unknown primary metastasis, due to diagnostic criteria overlapping.

A Case of Hepatocellular Carcinoma of Unknown Origin, Presenting as Huge Abdominal Mass in an 82-year-old Female Patient

Hepatocellular carcinoma(HCC) of unknown origin presents unusually in the elderly as a huge abdominal mass. Though most commonly associated with viral infections, about 10% of HCC in Korea arise from an unknown cause. HCC of unknown origin has a varying clinical picture and prognosis. It is known to have relatively good reserve liver function and rarely complications with previous cirrhosis or active hepatitis. Its pathogenesis may be not associated with an inflammation-dysplasia-carcinoma sequence. We recently managed an unusual case of moderately differentiated hepatocellular carinoma of unknown origin presenting with a huge abdominal mass. The 82-year-old female patient had no history of viral hepatitis and no other metabolic diseases were diagnosed. She received palliative care.

Dermatomyositis Associated with Cancer of Unknown Primary Site

Dermatomyositis (DM) is an uncommon inflammatory myopathy with characteristic rash accompanying, or more often preceding, muscle weakness. There is a well-recognized association between DM and several cancers, such as ovarian cancer, breast cancer, melanoma, colon cancer, and non-Hodgkin lymphoma. We report the first case of cancer of unknown primary site associated with DM. A 62-yr-old woman presented to us with both shoulder painful swelling and facial edema. She was diagnosed previously as cancer of unknown primary site, histologically confirmed with squamous cell carcinoma in a pelvic mass. For the following days, she complained of erythematous face followed by progressive weakness of the proximal muscles of upper and lower limbs. The laboratory tests showed an increased muscle enzyme and acute phase reactants. The electromyogram showed the typical findings of DM. After the treatment with high dose steroid and methotrexate, the proximal motor weakness improved, and she received palliative radiation therapy.