Leiomyoma With Massive Lymphoid Infiltration: 2 Cases With Lymphocytic Vasculopathy and Angiocentric Germinal Centers.

Uterine leiomyoma with massive lymphoid infiltration is characterized by a dense lymphoid infiltrate and germinal centers sparing the adjacent myometrium. Only few reports describe this entity and its etiology is unknown. This rare lesion may also exhibit lymphocytic vasculopathy but this has only been reported in the setting of GnRH agonist exposure. We report 2 cases of uterine leiomyoma with massive lymphoid infiltration in which only 1 patient was exposed to GnRH agonists. In both cases, histopathologic analysis showed thick-walled vessels with swollen endothelial cells showing evidence of intramural lymphocytic infiltration, red blood cell extravasation, and medial edema. This constellation of findings represented frank vascular damage and lymphocytic vasculopathy. Our findings suggest that lymphocytic vasculopathy in these lesions may be secondary to factors other than GnRH agonists. Furthermore, both cases showed an angiocentric disposition of germinal centers that has scarcely been alluded to in prior reports. This finding may provide a clue in accurately recognizing leiomyoma with massive lymphoid infiltration. Recognition of this lesion will allow one to avoid mistaking it for mimickers such as inflammatory myofibroblastic tumor, lymphoid malignancies, or other inflammatory processes.

Endosonography-Guided Biopsy as a First Test in the Diagnosis of Lymphoma.

To evaluate the diagnostic accuracy of Endobronchial Ultrasound-guided Transbronchial Needle Aspiration (EBUS-TBNA) and Endoscopic Ultrasound-guided Fine Needle Aspiration (EUS-FNA) in the diagnosis of lymphoma. A retrospective analysis of patients with suspected mediastinal lymphoproliferative disorders who underwent EBUS-TBNA, EUS-FNA or combined procedures from 2009 to 2019 was conducted using a prospectively maintained interventional thoracic endoscopy database. Demographic data, imaging, needle size, surgical biopsy, complications rate and pathology reports were reviewed. Over a 10-year period, a total of 444 patients were investigated with endosonography as the first diagnostic procedure for mediastinal adenopathy suspicious for lymphoma. Lymphoma was diagnosed in 77 patients (17.3%). In total, 68 patients (88.3%) were diagnosed using endosonographic mediastinal tissue sampling. Four patients had both lymphoproliferative disorders and lung cancer. Nine patients (11.7%) required a surgical biopsy to confirm the lymphoma diagnosis (6 non-diagnostic; 3 inadequate samples from endosonographic biopsies). In patients with adequate biopsies via endosonography, the sensitivity for the diagnosis of lymphoma, was 91.9% (n = 68/74). The histopathologic subtype of lymphoma was determined by endosonographic biopsies in 61 patients (89.7%) with an increased sensitivity (92.6%) for low grade Non-Hodgkin lymphoma (NHL). No acute complication related to endosonography was observed. Endosonographic biopsy (EBUS and/or EUS) of mediastinal adenopathy in patients with suspected lymphoma is a highly sensitive and safe diagnostic test. Endosonography should be the first test in the diagnosis of suspicious mediastinal lymphoma and should be followed by surgical biopsy in cases of insufficient sampling or indefinite diagnosis.

Case 285: Primary Breast Lymphoma.

History A 25-year-old woman was referred to our breast clinic for assessment of a palpable mass in her left breast that developed quickly in 2 weeks. She denied any associated fever, chills, redness, or pain. She had no relevant medical or surgical history; no evidence of recent pregnancy, abortion, or breastfeeding; and no family history of breast cancer. Clinical examination enabled confirmation of a firm mass occupying the retroareolar region and the outer quadrant of the left breast with no skin retraction, edema, or erythema. There was no evidence of enlarged axillary lymph nodes. US of the left breast, bilateral breast MRI, and fluorine 18 (18F) fluorodeoxyglucose (FDG) PET/CT were performed.

Pure erythroid leukemia.

Pure erythroid leukemia is a rare and aggressive form of acute leukemia with a deleterious clinical course. It is of erythroid lineage without myeloblastic component, representing >80% of marrow cellularity, with ≥30% proerythroblasts.

Case 285.

History A 25-year-old woman was referred to our breast clinic for assessment of a palpable mass in her left breast that developed quickly in 2 weeks. She denied any associated fever, chills, redness, or pain. She had no relevant medical or surgical history; no evidence of recent pregnancy, abortion, or breastfeeding; and no family history of breast cancer. Clinical examination enabled confirmation of a firm mass occupying the retroareolar region and the outer quadrant of the left breast with no skin retraction, edema, or erythema. There was no evidence of enlarged axillary lymph nodes. US of the left breast (Fig 1), bilateral breast MRI (Fig 2), and fluorine 18 fluorodeoxyglucose PET/CT (Fig 3) were performed.

Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass.

BACKGROUND: Erdheim-Chester disease is a rare histiocytic neoplasm associated with MAPK pathway mutations. Disease manifestation is variable often involving many different organs, mainly bone, retroperitoneum, the heart, and the central nervous system. Histological findings include foamy histiocytes in a fibrous stroma with scattered inflammatory infiltrate. Histiocytes are CD68 positive and S100 negative. Case Report. We report a case of Erdheim-Chester disease associated with small lymphocytic lymphoma presenting as a perirenal mass with a review of the recent literature. CONCLUSIONS: Erdheim-Chester disease rarely can be associated with other cancers, namely myeloid neoplasms. We report a case of Erdheim-Chester disease presenting with small lymphocytic lymphoma as a perirenal mass. The association of Erdheim-Chester disease with lymphoproliferative disorders needs to be elucidated.

Anisakiasis in a Canadian patient with incarcerated epigastric hernia.

Anisakiasis is human zoonotic parasitic infection caused by a nematode parasite called Anisakis. This infection is usually reported in Asian countries where consumption of raw seafood is common. Very few cases have been reported in North America. We present the case of a female Canadian patient with an Anisakis larvae in an incarcerated ventral hernia. Cases of Anisakis infections are exceedingly rare in western countries, with very few previous reports describing extra-gastrointestinal cases. Diagnosis is often difficult since the symptoms of anisakiasis are not pathognomonic. As the larvae cannot survive in the body, conservative treatment might be effective in intestinal anisakiasis and surgery is usually performed when complications are encountered. Preventive measures are crucial and include educating the public about the risks of raw fish consumption and the importance of visually inspecting consumed fish and freezing it before ingestion to kill the larvae and prevent the infection.

Severe immune mucositis and esophagitis in metastatic squamous carcinoma of the larynx associated with pembrolizumab.

BACKGROUND: Pembrolizumab is an anti-programmed death 1 (PD-1) receptor monoclonal antibody that has shown activity as second line treatment for metastatic head and neck squamous cell carcinoma (HNSCC). Immune-related adverse events are now well described complications of PD-1 inhibitors and most organ sites have been shown to be potentially affected. CASE PRESENTATION: We describe a 69-year old patient with a relapsed squamous cell carcinoma of the supraglottic larynx with lung metastasis after receiving adjuvant concurrent cisplatin and radiotherapy. This patient was treated with pembrolizumab and benefitted from therapy with major radiological improvement of disease. After 14 cycles of pembrolizumab 200 mg IV each 3 weeks, he experienced dysphagia that evolved to a grade 4 oral cavity and pharynx mucositis and esophagitis. Histologic analysis showed ulcerative esophagitis associated with granulation tissue. Pembrolizumab was discontinued and IV methylprednisolone 2 mg/kg/day was initiated. Two days later, the patient reported a 50% recovery in his symptoms which were completely resolved after 2 weeks. Methylprednisolone was switched to oral prednisone and a taper was planned over 8 weeks. During the fourth week of taper, the patient presented recurrence of grade 1 oral mucositis. Prednisone was increased 2 mg/kg/day for 2 weeks followed by slower tapering over a period of 5 months. Pembrolizumab was not reinitiated. CONCLUSION: This is the first described case of grade 4 immune mucositis and esophagitis associated with pembrolizumab. Because the use of pembrolizumab is increasing in oncology, pharmacists and physicians should be aware of this rare manifestation.

Coexistence of a choriocarcinoma and a gonadoblastoma in the gonad of a 46,XY female: a single nucleotide polymorphism array analysis.

Females with 46,XY complete gonadal dysgenesis are at significant risk of developing germ cell tumors, mostly gonadoblastomas. We present here the case of 2 half-sisters, sharing the same father, diagnosed with 46,XY complete gonadal dysgenesis. The 1st sister developed a gonadoblastoma and an invasive dysgerminoma, whereas the 2nd sister developed a gonadoblastoma and an invasive choriocarcinoma within the same gonad. No SRY mutation, chromosome abnormalities, or mosaicism were detected in blood. Single nucleotide polymorphism (SNP) profiling of the choriocarcinoma revealed a complex hyperdiploid pattern with gains of 1 to 4 copies of material from several autosomes, as well as the loss of the Y chromosome and a homozygous SNP profile without copy number change for the X chromosome. Our results are in agreement with the recurrent chromosome gains and losses previously published in germ cell tumors, and the coexistence of both tumors within the same gonad suggests that choriocarcinomas may derive from gonadoblastomas.