Late testicular relapse two decades after primary extragonadal germ cell tumor with uncommon metastases: a case report.

BACKGROUND: Extragonadal germ cell tumor (EGCT) is a relatively rare condition, reportedly representing 3-7% of all germ cell tumors. We report a patient who had metachronous testicular tumor with uncommon metastases 20 years after primary retroperitoneal EGCT treatment, along with a corresponding literature review. CASE PRESENTATION: A 49-year-old Japanese man visited our department in November 2017 with chief complaints of indolent right scrotum enlargement and a right inguinal mass. History showed that the patient visited our department of gastroenterology with chief complaints of blackish feces and ill complexion in February 1997. Computed tomography (CT) showed a right retroperitoneal tumor, which was removed in the same month. Histopathological examination showed a teratoma and yolk sac tumor. He was diagnosed with primary retroperitoneal EGCT and received three courses of chemotherapy (bleomycin/etoposide/cisplatin; BEP). Periodic imaging and the determination of tumor markers (alpha-fetoprotein [AFP], human chorionic gonadotropin [HCG], and lactate dehydrogenase [LDH]) showed no recurrence or metastasis during the 5 years postoperatively. Subsequently, he did not visit the outpatient ward. In August 1999, he underwent surgery of right hydrocele. Contrast-enhanced CT showed a 35-mm contrast effect with uneven content in the right testicle and enlarged nodes that raised suspicion for metastases in the right inguinal and right external iliac lymph nodes. All tumor markers were within normal ranges. He underwent right high orchiectomy and resection of the right inguinal lymph nodes in the same month. Histopathological findings revealed seminoma (pT1, pN2, M0, S0, and TNM stage IIB). He received postoperative chemotherapy, one course of BEP therapy, and three courses of etoposide and cisplatin therapy. Post-chemotherapy CT confirmed a complete clinical response at the right external iliac lymph nodes, and this response continued 12 months later. No recurrence or metastasis has been found so far. CONCLUSIONS: We report a patient in whom a testicular tumor with uncommon metastases occurred 20 years after primary retroperitoneal EGCT treatment. After EGCT treatment, testicular relapses tend to occur after relatively long-term follow-up. After EGCT treatment, such patients must be closely monitored for testicular recurrences and onset of testicular tumor.

[A Case of Metachronous Testicular Tumor Developing Twenty Years after Treatment of Retroperitoneal Extragonadal Germ Cell Tumor].

A 49-year-old male visited our department of gastroenterology with chief complaints of blackish feces and ill complexion in February 1997. Computed tomography (CT) revealed a right retroperitoneal tumor, which was removed the same month. Histopathological examination showed teratoma and yolk sac tumor. He was diagnosed with primary retroperitoneal extragonadal germ cell tumor, and received three cycles of chemotherapy (bleomycin/etoposide/cisplatin ; BEP) starting in March 1997. Periodic imaging and determination of tumor markers (α fetoprotein, human chorionic gonadotropin, and lactate dehydrogenase) showed no recurrence or metastasis for five years after treatment. After his visit in April 2002 he stopped visiting our outpatient ward. In November 2017, the patient visited our department with chief complaints of indolent right scrotum enlargement and a right inguinal mass. Past history showed that he had undergone hydrocele of the right testicle in August 1999. Contrast enhanced CT showed a 35-mm contrast effect with uneven contents in the right testis, and enlarged nodes that were suspicious of metastases in the right inguinal and right external iliac lymph nodes. All tumor markers were within the normal ranges. He underwent right high orchiectomy and resection of the right inguinal lymph nodes in the same month. Histopathological findings revealed seminoma (pT1, pN2, M0, S0, and clinical Stage IIA). He received postoperative chemotherapy starting in January 2018 ; one cycle of BEP therapy and three cycles of etoposide and cisplatin (EP) therapy. Post-chemotherapeutic CT confirmed clinical complete response at the right external iliac lymph nodes, and this response was confirmed 12 months later. Neither recurrence nor metastasis has occurred so far.

[A Case of Dedifferentiated Liposarcoma of the Spermatic Cord].

Liposarcomas are most commonly found in the extremities, in the retroperitoneum and, less often, in the head and neck area. The spermatic cord is a rare site of origin, accounting for about 4-7% of all liposarcomas. We report a case of dedifferentiated liposarcoma of the spermatic cord. A 51-year-old man was referred to our hospital for a painless hard mass in the left inguinal region. Abdominal computed tomography showed a left spermatic cord mass measuring 70 mm in diameter. We performed left high orchiectomy with resection of the mass. Immunohistochemical analysis revealed positive for murine double minute 2 (MDM 2) and cyclin dependent kinase 4 (CDK 4). Therefore, this sarcoma was diagnosed to be dedifferentiated liposarcoma. Since the surgical margin was positive, an additional wide resection including the surrounding normal tissue was performed. Complete excision was achieved after re-resection. He was alive 12 months postoperatively without any signs of recurrence. Dedifferentiated liposarcoma of the spermatic cord is a rare neoplasm. To the best of our knowledge, the present case is the 14th reported case in Japan.

Lung histopathological pattern in a survivor with rapidly progressive interstitial lung disease and anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis.

Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are specific indicators of patients with dermatomyositis, particularly clinically amyopathic dermatomyositis (CADM). CADM is occasionally accompanied by fatal, treatment-resistant, rapidly-progressive interstitial lung disease (RP-ILD). All previous reports showed that histopathological findings in RP-ILD with anti-MDA5 antibody-positive CADM indicated diffuse alveolar damage (DAD). This is the first report describing a non-DAD pattern in RP-ILD with anti-MDA5 antibody-positive CADM, which was improved by immunosuppressive therapy. This case may be a milder clinical phenotype than a typical DAD pattern in RP-ILD with anti-MDA5 antibody-positive CADM.

Adenocarcinoma arising in urinary bladder endocervicosis.

Endocervicosis is a rare benign condition characterized by the presence of endocervical-type mucinous glands. Urinary bladder endocervicosis forms an elevated lesion in the posterior wall of the urinary bladder and is sometimes misdiagnosed as a malignant tumor clinically and pathologically. Herein we describe the first case of adenocarcinoma arising in urinary bladder endocervicosis. The patient, a 58-year-old woman, presented with asymptomatic hematuria. Cystoscopy revealed a nodular mass measuring 4 cm in diameter in the posterior wall, and total cystectomy was performed. Histology revealed that the elevated lesion of the bladder wall was composed of haphazard proliferation of cystic glands lined by benign endocervical-type epithelium. An adenocarcinoma arose at the center of this endocervicosis. Mucin histochemistry revealed the presence of sulfomucin in both the endocervicosis and adenocarcinoma components. Immunohistochemically, the endocervicosis was positive for cytokeratin (CK) 7, AE1/AE3, CAM5.2, HBME1, CA19-9, and estrogen receptor (ER), and negative for CK20, CDX2, progesterone receptor (PR), MUC5AC, and ß-catenin. The adenocarcinoma showed similar immunohistochemical results, except for loss of ER expression and a slight increase in the ratio of Ki-67-positive cells. This case indicates that endocervicosis, known as a benign lesion, harbors the possibility of malignant transformation.

Efficacy of combined therapy with cyclosporin and low-dose prednisolone in interstitial pneumonia associated with connective tissue disease.

BACKGROUND: The optimal treatment method for interstitial pneumonia (IP) with connective tissue disease (CTD) remains controversial. In addition, a clinically meaningful end point to judge drug efficacy has not been fully investigated. OBJECTIVES: The aim of this study was to evaluate, from various aspects, the therapeutic benefit and tolerability of combined therapy with cyclosporin A (CsA) and low-dose prednisolone (PSL) for chronic fibrosing CTD-IP patients. METHODS: A total of 26 CTD-IP patients diagnosed by surgical lung biopsy and subsequently treated with the above combination therapy were retrospectively reviewed. The therapeutic regimen comprised methylprednisolone induction therapy for 2-4 weeks, followed by a combined therapy of CsA with low-dose PSL for 1 year. Evaluation of the therapeutic benefit was based on not only pulmonary function but also exercise capacity, health-related quality of life and dyspnea. RESULTS: After 1 year of therapy, clinically significant improvements in forced vital capacity (≥10%), carbon monoxide diffusing capacity of the lung (≥15%), 6-min walk distance (≥28 m), and St. George's Respiratory Questionnaire (≤-7) were observed in 61.5, 69.2, 61.5 and 69.2% of the patients, respectively. All measurements showed statistically significant improvements compared with baseline values. The 1-year treatment did not need to be discontinued in any patients due to unacceptable toxicity, and no deaths occurred. CONCLUSIONS: Combined therapy with CsA and low-dose PSL for CTD-IP patients was well-tolerated and patients displayed a noteworthy response.

Stinging in the oral cavity caused by ingestion of the sperm bags of a squid: a case report.

We present a case of stinging in the oral cavity caused by ingestion of the sperm bags of a squid. The patient experienced severe pain in her oral cavity immediately after eating raw squid. When she was examined at our hospital, we found that several small whitish spindle-shaped stings were stuck to the mucous membrane of the hard palate. A biopsy was performed, and the whitish stings were removed as well. We also performed a histological examination of the remaining part of the raw squid brought by the patient. The biopsy showed that the sperm bags of the squid had thrust into the squamous epithelium of the patient. The remaining part of the raw squid consisted of the testis and the sperm bags. After removal of all stings, the pain reduced, and the wound healed in due course. Larva migrans and anisakiasis are infections known to be caused by consumption of raw seafood. Although the condition reported here is relatively rare, doctors should also keep this condition in mind for patients reporting pain after eating raw seafood.

Serum KL-6 in fibrotic NSIP: Correlations with physiologic and radiologic parameters.

BACKGROUNDS: Fibrotic nonspecific interstitial pneumonia (f-NSIP) has been recognized as a distinct disease entity. KL-6 has been reported to be a useful serum marker in interstitial lung diseases. However, few previous reports evaluated the value of serum KL-6 exclusively in f-NSIP, as distinct from other subtypes of idiopathic interstitial pneumonia, therefore the associations of serum KL-6 with clinical and radiologic findings in this population remain unclear. METHODS: Serum KL-6 levels were measured in twenty-six consecutive patients with f-NSIP diagnosed by surgical lung biopsy. Pulmonary function testing, bronchoalveolar lavage, subjective measurement of dyspnea using baseline dyspnea index (BDI), and HRCT were performed in parallel. Two radiologists conducted independent visual examinations of the pattern and extent of abnormalities on HRCT. RESULTS: Serum KL-6 levels were elevated above the cut-off level in all patients. In univariate analysis serum KL-6 levels showed negative correlations with BDI (rho=-0.52; p<0.01). Serum KL-6 had positive correlations with the extent of several patterns of opacities (rho=0.56-0.62; p<0.01). Among them, only the extent of traction bronchiectasis in HRCT showed significant association with serum KL-6 in multivariate analysis (beta-coefficient=0.043; p<0.01). CONCLUSIONS: Serum levels of KL-6 were elevated in f-NSIP, and were correlated with the extent of fibrotic abnormalities on HRCT, suggesting a value of serum KL-6 as a marker for fibrosis in f-NSIP.

Epithelial inclusion cyst arising in an intramammary lymph node: case report with cytologic findings.

The occurrence of epithelial inclusion cysts (EIC) in axillary lymph nodes is a rare but well recognized entity, arising either from direct implantation or from embryonal rests. Theoretically, EIC can occur in intramammary lymph nodes, but there has been only one prior report of such a lesion. Here, we describe a case of an EIC arising in an intramammary lymph node of a 37-year-old woman. This report focuses on the FNA cytologic features of this lesion and its differential diagnoses. On FNA, the EIC arising in an intramammary lymph node was characterized by mature lymphocytes, squamous epithelial cells, and keratinizing material. The presence of squamous cells can lead to the erroneous diagnosis of more common breast lesions, such as squamous cell carcinoma or metaplastic carcinoma. Contrary to these more sinister diagnoses, EIC arising in an intramammary lymph node is a benign condition. As this rare lesion sometimes mimics a neoplasm both clinically and radiographically, awareness of this entity is important to prevent over treatment.

Nestin expression as a new marker in malignant peripheral nerve sheath tumors.

Malignant peripheral nerve sheath tumor (MPNST) can be difficult to diagnose because it lacks specific immunohistochemical markers. S-100, which is a useful marker of MPNST, has limited diagnostic utility. Recent studies suggest that nestin, which is an intermediate filament protein, is expressed in neuroectodermal stem cells. The diagnostic utility of immunostains for nestin and three other neural markers (S-100, CD56 and protein gene product 9.5 (PGP 9.5)) were evaluated in 35 cases of MPNST and in other spindle cell tumors. All MPNST cases were strongly positive for nestin and had cytoplasmic staining. Stains for S-100, CD56, and PGP 9.5 were positive in fewer cases (17/35, 11/35, and 29/35 cases, respectively), and had less extensive staining. Nestin was negative in 10/10 leiomyomas, and weak nestin expression was seen in 10/10 schwannomas, 3/10 neurofibromas, 2/8 synovial sarcomas, 2/10 liposarcomas, 4/7 carcinosarcomas and 3/7 malignant fibrous histiocytomas. In contrast, strong nestin positivity was seen in 10/10 rhabdomyosarcomas, 15/19 leiomyosarcomas, and 9/9 desmoplastic melanomas. Nestin is more sensitive for MPNST than other neural markers and immunostains for nestin in combination with other markers could be useful in the diagnosis of MPNST.

Malignant mesothelioma of the tunica vaginalis testis: a case with a predominant sarcomatous component.

We present a case of malignant mesothelioma (MM) of the tunica vaginalis testis. A 64-year-old man was referred for an operation on a right hydrocele that later proved to be a tumor during surgery. The tumor was malignant with a biphasic pattern of epithelial and sarcomatous components. The latter component was predominant. Cuboidal or columnar cells formed irregular tubular structures in the epithelial component. In contrast, spindle-shaped or polygonal cells formed intricate structures with stromal connective tissues in the sarcomatous component. Immunohistochemical staining revealed that the tumor was mesothelial in origin and positive for cytokeratin, vimentin, HBME-1 antigen and calretinin. In general, MM occur in the pleura or peritoneum; those originating in the tunica vaginalis testis are very rare and represent less than 5% of all MM. In addition, MM in the tissues usually consist primarily of an epithelial component. According to previous reports tumors with a predominant sarcomatous component are extremely rare. In general, a sarcomatous component predicts poor prognosis and our case does, in fact, deteriorate over time. Our case suggests that despite its low incidence, MM must be considered when a case is diagnosed as hydrocele testicle.