Recurrent Submandibular Gland Swelling Relieved by Antihistamine Medication: Is It Eosinophilic Sialodochitis? / 대한이비인후과학회지

Sialodochitis fibrinosa is a disease characterized by salivary duct obstruction. A 21-year-old male presented with a painful submandibular gland (SMG) swelling. Serum eosinophilia and Whartons’ duct dilatation with strong enhancement were observed on contrast CT. Core needle biopsy (CNB) for SMG parenchyma revealed lymphocytic infiltration between dilated intralobular ducts. In another case, a 39-year-old male complained of recurrent major salivary glands swelling for ten years with an itching sensation on the overlying skin of the salivary gland. Enhancement of both SMG parenchyma, dilatation of both Wharton’s ducts and elevated serum eosinophilia were observed on contrast CT study. CNB for SMG parenchyma revealed lymphocytic infiltration with many eosinophils around a markedly dilated interlobular duct. The recurrent SMG swelling in both cases were relieved by antihistamine medication, warranting suspicion that these cases might organ-specific eosinophilic disease. We dicuss these two cases with a literature review.

Osteoma Cutis on Eyelid

PURPOSE@#To present the first report describing lesions of osteoma cutis on the upper eyelid and medial canthus.CASE SUMMARY: A 4-year-old female complained of a right upper eyelid mass. The examination showed a well-delineated, mild bluish-colored, hard mass over the upper eyelid and the medial canthus measuring 10 × 10 mm and 2 × 2 mm. During the cutaneous examination, her forearm, left shin, right dorsum of the foot, neck, and abdominal wall also showed well-delineated, mild bluish-colored, immobile hard masses, similar to the upper eyelid mass. A right upper eyelid and medial canthus mass excision was performed and a biopsy specimen was collected. Hematoxyline and eosin staining showed a mature bone in the dermis with spicules of bone and osteoblasts. She was finally diagnosed with osteoma cutis on the upper eyelid and the medial canthus.@*CONCLUSIONS@#Osteoma cutis is a rare, benign skin disorder characterized by bone formation in the skin. Albright hereditary osteodystrophy, which causes a metabolic disorder, should also be considered to prevent complications.

CRISPR/Cas9-mediated knockout of CD47 causes hemolytic anemia with splenomegaly in C57BL/6 mice / 한국실험동물학회지

CD47 (integrin-associated protein), a multi-spanning transmembrane protein expressed in all cells including red blood cells (RBCs) and leukocytes, interacts with signal regulatory protein α (SIRPα) on macrophages and thereby inhibits phagocytosis of RBCs. Recently, we generated a novel C57BL/6J CD47 knockout (CD47(−/−) hereafter) mouse line by employing a CRISPR/Cas9 system at Center for Mouse Models of Human Disease, and here report their hematological phenotypes. On monitoring their birth and development, CD47(−/−) mice were born viable with a natural male-to-female sex ratio and normally developed from birth through puberty to adulthood without noticeable changes in growth, food/water intake compared to their age and sex-matched wild-type littermates up to 26 weeks. Hematological analysis revealed a mild but significant reduction of RBC counts and hemoglobin in 16 week-old male CD47(−/−) mice which were aggravated at the age of 26 weeks with increased reticulocyte counts and mean corpuscular volume (MCV), suggesting hemolytic anemia. Interestingly, anemia in female CD47(−/−) mice became evident at 26 weeks, but splenomegaly was identified in both genders of CD47(−/−) mice from the age of 16 weeks, consistent with development of hemolytic anemia. Additionally, helper and cytotoxic T cell populations were considerably reduced in the spleen, but not in thymus, of CD47(−/−) mice, suggesting a crucial role of CD47 in proliferation of T cells. Collectively, these findings indicate that our CD47(−/−) mice have progressive hemolytic anemia and splenic depletion of mature T cell populations and therefore may be useful as an in vivo model to study the function of CD47.

Delayed formation of sterile abscess after zygomaticomaxillary complex fracture treatment with bioabsorbable plates

We present a patient who showed a sterile abscess after facial bone fixation with bioabsorbable plates and screws. He had zygomaticomaxillary complex and periorbital fracture due to falling down. The displaced bones were treated by open reduction and internal fixation successfully using bioabsorbable plate system. However, at postoperative 11 months, abrupt painless swelling was noted on the previous operation sites, left lateral eyebrow and lower eyelid. By surgical exploration, pus-like discharge and degraded materials were observed and debrided. The pathologic analysis revealed foreign body reaction with sterile abscess. This complication followed by bioabsorbable device implantation on maxillofacial bone surgery has been rarely reported in which we call attention to the maxillofacial plastic surgeons.

A Case of Neutrophilic Myositis

Neutrophilic myositis is a very rare disease histologically characterized by neutrophil infiltration of muscle tissues. We report a case of a 47-year-old man who presented with acute onset of severe swelling and pain on his left shoulder with high fever. He was initially suspected of having cellulitis, but intravenous antibiotics did not improve his symptoms. Similar swelling and pain then developed on both calves. Investigations with magnetic resonance imaging of the lower legs and muscle biopsy led to a diagnosis of neutrophilic myositis. High dose glucocorticoid dramatically improved his symptoms within days. Clinicians need to be aware of this rare disease as a cause of acute febrile myositis mimicking infection.

Intravascular Large B-cell Lymphoma within Duodenal Gastrointestinal Stromal Tumor / 대한췌담도학회지

Intravascular large B-cell lymphoma (IVLBCL) is a rare disease of intravascular growth of malignant lymphocytes without an obvious extravascular tumor mass or existence in peripheral blood. It has poor prognosis due to its aggressive behavior and rapid systemic dissemination. But there is no pathognomonic finding, diagnosis of IVLBCL is still challenging. Here we report a case of IVLBCL found within a resected specimen of duodenal gastrointestinal stromal tumor.

Intravascular Large B-cell Lymphoma within Duodenal Gastrointestinal Stromal Tumor / 대한췌담도학회지

Intravascular large B-cell lymphoma (IVLBCL) is a rare disease of intravascular growth of malignant lymphocytes without an obvious extravascular tumor mass or existence in peripheral blood. It has poor prognosis due to its aggressive behavior and rapid systemic dissemination. But there is no pathognomonic finding, diagnosis of IVLBCL is still challenging. Here we report a case of IVLBCL found within a resected specimen of duodenal gastrointestinal stromal tumor.

Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a slowly progressive neoplastic disease that predominantly affects females. Usually, LAM affects the lung; it can also affect extrapulmonary sites, such as the mediastinum, the retroperitoneum, or the lymph nodes, although these locations are rare. A localized form of LAM can manifest as extrapulmonary lesions; this form is referred to as extrapulmonary lymphangioleiomyoma (E-LAM). Due to the rare occurrence of E-LAM and its variable, atypical location, E-LAM is often difficult to diagnose. Herein, we report the clinicopathological information from four E-LAM cases, and also review previous articles investigating this disease. METHODS: Four patients with E-LAM were identified at the Samsung Medical Center (Seoul, Korea) from 1995 to 2012. All E-LAM lesions underwent surgical excision. RESULTS: All patients were females within the age range of 43 to 47 years. Two patients had para-aortic retroperitoneal masses, while the other two patients had pelvic lesions; two out of the four patients also had accompanying pulmonary LAM. In addition, no patient displayed any evidence of tuberous sclerosis. Histologically, two patients exhibited nuclear atypism with cytologic degeneration. CONCLUSIONS: E-LAM should be considered in the differential diagnosis of patients presenting with pelvic or para-aortic masses. We also conclude that further clinical and pathological evaluation is needed in patients with E-LAM and nuclear atypism.

Usual Interstitial Pneumonia with Lung Cancer: Clinicopathological Analysis of 43 Cases

BACKGROUND: Previous studies have suggested an association between usual interstitial pneumonia (UIP) and lung cancer (Ca). However, clinical and histological information is not enough to determine such an association, due to the low incidence and short survival time of patients with both conditions. METHODS: We retrospectively reviewed the clinical and histological records of Ca patients with UIP between January 1999 and August 2013 at the Samsung Medical Center, Seoul, Korea. We found 43 patients who had Ca with UIP (UIP-Ca). Previously reported data of eighty-four patients with UIP-only were included as a comparison group. RESULTS: Smoking is related to poor prognosis in patients with UIP-Ca, and the number of patients with a high smoking index of more than 30 pack-years significantly increased in UIP-Ca patients compared with UIP-only patients. There is no significant prognostic differentiation between UIP-Ca patients and UIP-only patients. Microscopically, UIP-Ca patients showed characteristically heterogeneous histological patterns and degrees of differentiation. There were many foci of squamous metaplasia or dysplasia at the peripheral area of squamous cell carcinomas. CONCLUSIONS: We report 43 cases of UIP-Ca. Our results suggest that smoking is related to cancer occurrence in UIP patients and poor prognosis in UIP-Ca patients.

Primary Follicular Lymphoma in a Male Breast: A Case Report / Journal of the Korean Cancer Association, 대한암학회지

Primary breast lymphoma (PBL) is a rare disease, particularly in males. Diffuse large B cell lymphoma is the most common PBL, while follicular lymphoma is less common. Furthermore, primary follicular lymphoma of a male breast is rarely reported. We report a male patient with primary follicular lymphoma of the breast and hepatocellular carcinoma (HCC). A 46-year-old man was diagnosed with liver cirrhosis secondary to chronic hepatitis B infection. Ten years later, he underwent segmentectomy of the liver due to HCC. Another 5 months later, he presented with a painless mass in the right chest wall. The mass was diagnosed as follicular lymphoma of the breast. The stage was IEA and he did not receive adjuvant therapy. Although only a few cases have been reported, lymphoma should be considered as a possible cause of breast mass, even in male patients.

Primary Follicular Lymphoma in a Male Breast: A Case Report / Journal of the Korean Cancer Association, 대한암학회지

Primary breast lymphoma (PBL) is a rare disease, particularly in males. Diffuse large B cell lymphoma is the most common PBL, while follicular lymphoma is less common. Furthermore, primary follicular lymphoma of a male breast is rarely reported. We report a male patient with primary follicular lymphoma of the breast and hepatocellular carcinoma (HCC). A 46-year-old man was diagnosed with liver cirrhosis secondary to chronic hepatitis B infection. Ten years later, he underwent segmentectomy of the liver due to HCC. Another 5 months later, he presented with a painless mass in the right chest wall. The mass was diagnosed as follicular lymphoma of the breast. The stage was IEA and he did not receive adjuvant therapy. Although only a few cases have been reported, lymphoma should be considered as a possible cause of breast mass, even in male patients.

Primary breast lymphoma: a single institution's experience / 대한외과학회지

PURPOSE: Primary breast lymphoma is a very rare disease, accounting for 0.4-0.5% of all breast malignancies. Due to the rarity, there are only limited reports of this disease in Korean women. In this reason, we report the experience of a single institution in Korea with primary breast lymphoma (PBL). METHODS: We retrospectively reviewed the medical records of 9 patients with PBL and evaluated the clinicopathologic characteristics and treatment outcomes. RESULTS: All nine patients were female and had diffuse large B-cell lymphoma (DLBL). The median age at diagnosis was 47.9 years and the median tumor size was 3.8 cm in diameter. The most common symptom was a painless palpable mass. Five patients were classified as stage IEA and four patients were IIEA according to the Ann Arbor staging system. Four patients underwent excisional biopsy and one patient underwent a lumpectomy with sentinel lymph node biopsy due to uncertain histology of the preoperative core needle biopsy. Nine patients received anthracycline containing combined chemotherapy; among them, five patients were treated with a rituximab containing regimen. Four patients received radiotherapy combined with chemotherapy. A complete response was achieved in eight patients. During the 44 months of the median follow-up period, three cases of relapse occurred, and among them, two patients died due to disease progression. CONCLUSION: Most PBLs are B-cell origin, with DLBL being the most common histologic type. A combined treatment modality has been known to have positive effects on prognosis, and surgery should be limited to a diagnostic purpose.

Breast Metastasis from Rhabdomyosarcoma of the Anus in an Adolescent Female / 한국유방암학회지

Rhabdomyosarcoma (RMS) of the breast is rare and there is scant information about the clinical behavior and treatment strategies. We report an adolescent female patient with metastatic RMS of the breast from the anus. An 18-year-old female patient was referred to our clinic due to palpable mass in the left breast. At age seven, she was diagnosed with acute lymphoblastic leukemia and treated with chemoradiation therapy. After 10 years of complete remission state, she presented with anal mass which was diagnosed as RMS and she received chemoradiation therapy. After 1 year of complete remission state, she noticed a palpable mass in her left breast. The breast mass was diagnosed as metastatic RMS based on core needle biopsy specimen. The RMS in breast was excised for the decreasing tumor burden despite of another metastatic lesion. Although rarely reported, metastasis of RMS should be considered as a cause of breast mass. Tissue biopsy is recommended when clinically suspected lesion is detected.

Fine Needle Aspiration Cytology of Kikuchi's Lymphadenitis: with Emphasis on Differential Diagnosis with Tuberculosis

BACKGROUND: Although Kikuchi's lymphadenitis (KL) has been known to have characteristic cytological features, pathologists encounter difficulties in making a diagnosis with fine needle aspiration cytology (FNAC). The objective of this study was to assess the diagnostic pitfalls of KL with FNAC, particularly with emphasis on differential diagnosis with tuberculosis. METHODS: FNAC of 10 patients with a histological diagnosis of KL and tuberculosis was reviewed. RESULTS: Acidophilic cells were observed in all the 10 KL cases, even if the smears were insufficient. Crescentic histiocytes were seen in 8, granular background in 7, and karyorrhectic debris in 3 cases. Epithelioid histiocytes or neutrophils were not seen in any of the KL cases. Of the 10 cases of tuberculosis, acidophilic cells were observed in 6 cases, crescentic histiocytes in none of them, cheese-like background in 9, karyorrhectic debris in 8, epithelioid histiocytes in 4, and neutrophils in 8 cases. CONCLUSIONS: The acidophilic cell could be the most sensitive but not the specific marker of KL with FNAC. The crescentic histiocytes might be the sensitive and considerably specific marker of KL. The cytological features distinguishing tuberculosis from KL may be cheese-like necrosis admixed with neutrophils and epithelioid histiocytes.

Aspiration Cytology of the Osteoclastic Variant of Anaplastic Thyroid Carcinoma: with Special Emphasis on the Undifferentiated Mononuclear Cells

Anaplastic thyroid carcinoma (ATC) is an uncommon aggressive malignant tumor, and the osteoclastic variant of ATC is extremely rare. We report here on the fine needle aspiration cytology of the osteoclastic variant of ATC in an 83-year-old woman. The smear was composed of many oval to slightly elongated undifferentiated mononuclear cells admixed with multinucleated osteoclast-like giant cells. The mononuclear tumor cells revealed inconspicuous nuclear pleomorphism and the nuclei were characterized by vesicular chromatin and an indented or lobulated nuclear membrane with conspicuous nuclear grooves. A few epithelial clusters suggestive of a papillary carcinoma component were also observed. Making the proper cytological diagnosis of the osteoclastic variant of ATC is helpful to determine the proper treatment modality for these patients.

Fine Needle Aspiration Cytology of Pulmonary Epithelioid Hemangioendothelioma with Prominent Hyaline Degeneration: A Case Report

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of low to intermediate malignant potential, and PEH can mimic other more common tumor entities pathologically as well as clinically. Compared to its well-recognized histological features, its cytological findings have been reported rarely to be plasmacytoid or epithelioid cells with abundant dense or finely granular cytoplasm, cytoplasmic vacuoles, round nuclei and prominent nucleoli.We report here on the fine needle aspiration cytologic findings of a 38-year-old woman with EH of the lung, that showed in addition to its classical cytomorphology, a somewhat peculiar cytologic finding such as big twig-like rosettoid structures with prominent hyalinized stroma. This tumor was histologically and immunohistochemically proven to be PEH by primary antibodies for CD31, CD34 and vimentin. We emphasize that the accuracy of making a cytologic diagnosis of this rare tumor can be increased by recognizing the peculiar cytologic finding that we report on here.

Fine Needle Aspiration Cytology of Pulmonary Epithelioid Hemangioendothelioma with Prominent Hyaline Degeneration: A Case Report

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of low to intermediate malignant potential, and PEH can mimic other more common tumor entities pathologically as well as clinically. Compared to its well-recognized histological features, its cytological findings have been reported rarely to be plasmacytoid or epithelioid cells with abundant dense or finely granular cytoplasm, cytoplasmic vacuoles, round nuclei and prominent nucleoli.We report here on the fine needle aspiration cytologic findings of a 38-year-old woman with EH of the lung, that showed in addition to its classical cytomorphology, a somewhat peculiar cytologic finding such as big twig-like rosettoid structures with prominent hyalinized stroma. This tumor was histologically and immunohistochemically proven to be PEH by primary antibodies for CD31, CD34 and vimentin. We emphasize that the accuracy of making a cytologic diagnosis of this rare tumor can be increased by recognizing the peculiar cytologic finding that we report on here.

A Case of Intestinal Duplication Cyst Identified as Pathological Lead Point in a Child with Recurrent Intussusception / 대한소아소화기영양학회지

Intestinal duplication cysts are characterized by the attachment to some part of the gastrointestinal tract with which a blood supply is shared, and have an epithelial lining resembling some part of the alimentary tract. A 15-month-old female was admitted to our hospital with cyclic irritability, vomiting, and blood-tinged stool. The results of an ultrasound showed an ileocolic intussusception and a 1.3 cm cystic mass had double-wall sign and a Y-configuration with an adjacent ileal loop. She had a past history of two ileocolic intussusceptions. The cystic mass was considered to be a pathologic lead point, so resection and end-to-end anastomosis was performed. The gross and histologic evaluation of the specimen demonstrated a 2.4x2.4 cm cystic mass containing yellow mucoid fluid and the cyst wall was lined with intestinal and gastric mucosa and enclosed by a layer of muscle, which was shared with the adjacent ileum.